Simultaneous presence of two hematological malignancies: chronic lymphocytic leukemia and myelofibrosis in a patient.

PubMed

Palta, Anshu; Garg, Shailja; Chauhan, Sandeep; Varma, Neelam

2011-03-01

Coexistence of chronic lymphocytic leukemia (CLL) with myelofibrosis is a rare association with only isolated case reports in the literature. We report an unusual case of CLL in which the cause of anemia was coexistent myelofibrosis. In a case of CLL presenting with refractory anemia, besides common causes like autoimmune hemolytic anemia and marrow infiltration, other causes like myelofibrosis should be searched for.

Cytogenetics, JAK2 and MPL mutations in polycythemia vera, primary myelofibrosis and essential thrombocythemia

PubMed Central

dos Santos, Leonardo Caires; Ribeiro, Juliana Corrêa da Costa; Silva, Neusa Pereira; Cerutti, Janete; da Silva, Maria Regina Regis; Chauffaille, Maria de Lourdes Lopes Ferrari

2011-01-01

Background The detection of molecular and cytogenetic alterations is important for the diagnosis, prognosis and classification of myeloproliferative neoplasms. Objectives The aim of this study was to detect the following mutations: JAK2 V617F, JAK2 exon 12 and MPL W515K/L, besides chromosomal abnormalities. Furthermore, molecular and cytogenetic alterations were correlated with the leukocyte and platelet counts, hemoglobin levels and age in all patients and with the degree of fibrosis in primary myelofibrosis cases. Methods Twenty cases of polycythemia vera, 17 of essential thrombocythemia and 21 of primary myelofibrosis were selected in the Hematology Department of the Universidade Federal de São Paulo (UNIFESP) between February 2008 and December 2009. The JAK2 V617F, JAK2 exon 12 mutations, MPL W515K and MPL W515L mutations were investigated by real-time PCR and direct sequencing. G-band karyotyping and fluorescence in situ hybridization were used to detect chromosomal abnormalities. Results Chromosomal abnormalities were observed only in polycythemia vera (11.8%) and primary myelofibrosis cases (17.6%), without correlation to clinical data. Chromosomal abnormalities were not detected by fluorescence in situ hybridization. The JAK2 V617F mutation was observed in polycythemia vera (90%), primary myelofibrosis (42.8%) and essential thrombocythemia (47%). Patients with JAK2 V617F-negative polycythemia vera had lower platelet and leukocyte counts compared to V617F-positive polycythemia vera (p-value = 0.0001 and p-value = 0.023, respectively). JAK2 V617F-positive and MPL W515L-positive primary myelofibrosis cases had a higher degree of fibrosis than V617F-negative cases (p-value = 0.022). JAK2 exon 12 mutations were not detected in polycythemia vera patients. The MPL W515L mutation was observed in one case of primary myelofibrosis and in one of essential thrombocythemia. The MPL W515K mutation was not found in patients with essential thrombocythemia or primary

Cytogenetics, JAK2 and MPL mutations in polycythemia vera, primary myelofibrosis and essential thrombocythemia.

PubMed

Dos Santos, Leonardo Caires; Ribeiro, Juliana Corrêa da Costa; Silva, Neusa Pereira; Cerutti, Janete; da Silva, Maria Regina Regis; Chauffaille, Maria de Lourdes Lopes Ferrari

2011-01-01

The detection of molecular and cytogenetic alterations is important for the diagnosis, prognosis and classification of myeloproliferative neoplasms. THE AIM OF THIS STUDY WAS TO DETECT THE FOLLOWING MUTATIONS: JAK2 V617F, JAK2 exon 12 and MPL W515K/L, besides chromosomal abnormalities. Furthermore, molecular and cytogenetic alterations were correlated with the leukocyte and platelet counts, hemoglobin levels and age in all patients and with the degree of fibrosis in primary myelofibrosis cases. Twenty cases of polycythemia vera, 17 of essential thrombocythemia and 21 of primary myelofibrosis were selected in the Hematology Department of the Universidade Federal de São Paulo (UNIFESP) between February 2008 and December 2009. The JAK2 V617F, JAK2 exon 12 mutations, MPL W515K and MPL W515L mutations were investigated by real-time PCR and direct sequencing. G-band karyotyping and fluorescence in situ hybridization were used to detect chromosomal abnormalities. Chromosomal abnormalities were observed only in polycythemia vera (11.8%) and primary myelofibrosis cases (17.6%), without correlation to clinical data. Chromosomal abnormalities were not detected by fluorescence in situ hybridization. The JAK2 V617F mutation was observed in polycythemia vera (90%), primary myelofibrosis (42.8%) and essential thrombocythemia (47%). Patients with JAK2 V617F-negative polycythemia vera had lower platelet and leukocyte counts compared to V617F-positive polycythemia vera (p-value = 0.0001 and p-value = 0.023, respectively). JAK2 V617F-positive and MPL W515L-positive primary myelofibrosis cases had a higher degree of fibrosis than V617F-negative cases (p-value = 0.022). JAK2 exon 12 mutations were not detected in polycythemia vera patients. The MPL W515L mutation was observed in one case of primary myelofibrosis and in one of essential thrombocythemia. The MPL W515K mutation was not found in patients with essential thrombocythemia or primary myelofibrosis. The MPL W515L

Multiple esophageal variceal ruptures with massive ascites due to myelofibrosis-induced portal hypertension

PubMed Central

Tokai, Koichi; Miyatani, Hiroyuki; Yoshida, Yukio; Yamada, Shigeki

2012-01-01

A 75-year old man had been diagnosed at 42 years of age as having polycythemia vera and had been monitored at another hospital. Progression of anemia had been recognized at about age 70, and the patient was thus referred to our center in 2008 where secondary myelofibrosis was diagnosed based on bone marrow biopsy findings. Hematemesis due to rupture of esophageal varices occurred in January and February of 2011. The bleeding was stopped by endoscopic variceal ligation. Furthermore, in March of the same year, hematemesis recurred and the patient was transported to our center. He was in irreversible hemorrhagic shock and died. The autopsy showed severe bone marrow fibrosis with mainly argyrophilic fibers, an observation consistent with myelofibrosis. The liver weighed 1856 g the spleen 1572 g, indicating marked hepatosplenomegaly. The liver and spleen both showed extramedullary hemopoiesis. Myelofibrosis is often complicated by portal hypertension and is occasionally associated with gastrointestinal hemorrhage due to esophageal varices. A patient diagnosed as having myelofibrosis needs to be screened for esophageal/gastric varices. Myelofibrosis has a poor prognosis. Therefore, it is necessary to carefully decide the therapeutic strategy in consideration of the patient’s concomitant conditions, treatment invasiveness and quality of life. PMID:22851873

Phase II Evaluation of IPI-926, an Oral Hedgehog Inhibitor, in Patients with Myelofibrosis

PubMed Central

Sasaki, Koji; Gotlib, Jason R.; Mesa, Ruben A.; Newberry, Kate J.; Ravandi, Farhad; Cortes, Jorge E.; Kelly, Patrick; Kutok, Jeffery L.; Kantarjian, Hagop M.; Verstovsek, Srdan

2016-01-01

The clinical safety and efficacy of IPI-926 was evaluated in 14 patients with myelofibrosis in a phase II study. Patients received 160-mg IPI-926 orally in continuous 28-day cycles. The median treatment duration was 5.1 months, and all patients had discontinued treatment by 7.5 months. Nine patients discontinued due to lack of response as determined by the treating physician, two after developing acute leukemia and one due to disease progression/loss of response. Twelve patients had slight reductions in spleen size (less than 50% from baseline), but symptoms did not improve consistently. One patient achieved transfusion independence lasting 5 months. Reductions in GLI1 mRNA and protein levels, JAK2V617F allele burden, degree of fibrosis, or cytokine levels were observed in some patients, but were not significant when evaluated for the cohort. Low-grade gastrointestinal/liver abnormalities were the most common toxicities. The results did not support continued evaluation of IPI-926 as a monotherapy in myelofibrosis. PMID:25641433

Ferrokinetic study of splenic erythropoiesis: Relationships among clinical diagnosis, myelofibrosis, splenomegaly, and extramedullary erythropoiesis

DOE Office of Scientific and Technical Information (OSTI.GOV)

Beguin, Y.; Fillet, G.; Bury, J.

1989-10-01

Splenic erythropoiesis was demonstrated by surface counting of {sup 59}Fe in 129 of 1,350 ferrokinetic studies performed over a 15 year period. These 129 studies were carried out in 108 patients, including 40 with chronic myelogenous leukemia (CML), 24 with agnogenic myeloid metaplasia (AMM), 18 with polycythemia vera (PV), six with a myelodysplastic syndrome, five with acute leukemia, three with prostate or breast carcinoma, two each with aplastic anemia or Hodgkin's disease, and one each with idiopathic thrombocythemia, multiple myeloma, chronic renal failure, or treated hypopituitarism. Splenomegaly was present in 83% of the studies and hepatomegaly in 72%. Grade II-IIImore » myelofibrosis was demonstrated in 62% of the cases. Hepatic erythropoiesis was present in 77% of the studies (only 38% in PV), and marrow erythropoiesis was undetectable in 33%. Total erythropoiesis was about twice normal (range 0.2 to 8 times normal) but was ineffective to varying degrees in 86% of the studies. Relationships between organomegaly, myelofibrosis, and extramedullary erythropoiesis, as well as differences among clinical disorders, are discussed. Differences observed between CML in chronic or blastic phase suggested that the erythroid cell line was involved in the proliferative process. It is concluded that splenic erythropoiesis (1) is encountered in a variety of clinical conditions; (2) is not necessarily associated with splenomegaly or myelofibrosis, even in the myeloproliferative disorders; (3) is part of a predominantly extramedullary (in the liver as well as in the spleen), expanded, and largely inefficient total erythropoiesis; and (4) can be evaluated in a semiquantitative manner by surface counting.« less

Mesenchymal Cell Reprogramming in Experimental MPLW515L Mouse Model of Myelofibrosis.

PubMed

Han, Ying; Yue, Lanzhu; Wei, Max; Ren, Xiubao; Shao, Zonghong; Zhang, Ling; Levine, Ross L; Epling-Burnette, Pearlie K

2017-01-01

Myelofibrosis is an indicator of poor prognosis in myeloproliferative neoplasms (MPNs), but the precise mechanism(s) contributing to extracellular matrix remodeling and collagen deposition in the bone marrow (BM) niche remains unanswered. In this study, we isolated mesenchymal stromal cells (MSCs) from mice transplanted with wild-type thrombopoietin receptor (MPLWT) and MPLW515L retroviral-transduced bone marrow. Using MSCs derived from MPLW515-transplant recipients, excessive collagen deposition was maintained in the absence of the virus and neoplastic hematopoietic cells suggested that the MSCs were reprogrammed in vivo. TGFβ production by malignant megakaryocytes plays a definitive role promoting myelofibrosis in MPNs. However, TGFβ was equally expressed by MSCs derived from MPLWT and MPLW515L expressing mice and the addition of neutralizing anti-TGFβ antibody only partially reduced collagen secretion in vitro. Interestingly, profibrotic MSCs displayed increased levels of pSmad3 and pSTAT3 suggesting that inflammatory mediators cooperating with the TGFβ-receptor signaling may maintain the aberrant phenotype ex vivo. FGFb is a known suppressor of TGFβ signaling. Reduced collagen deposition by FGFb-treated MSCs derived from MPLW515L mice suggests that the activating pathway is vulnerable to this suppressive mediator. Therefore, our findings have implications for the future investigation of therapies to reverse fibrosis in MPNs.

Mesenchymal Cell Reprogramming in Experimental MPLW515L Mouse Model of Myelofibrosis

PubMed Central

Wei, Max; Ren, Xiubao; Shao, Zonghong; Zhang, Ling; Levine, Ross L.; Epling-Burnette, Pearlie K.

2017-01-01

Myelofibrosis is an indicator of poor prognosis in myeloproliferative neoplasms (MPNs), but the precise mechanism(s) contributing to extracellular matrix remodeling and collagen deposition in the bone marrow (BM) niche remains unanswered. In this study, we isolated mesenchymal stromal cells (MSCs) from mice transplanted with wild-type thrombopoietin receptor (MPLWT) and MPLW515L retroviral-transduced bone marrow. Using MSCs derived from MPLW515-transplant recipients, excessive collagen deposition was maintained in the absence of the virus and neoplastic hematopoietic cells suggested that the MSCs were reprogrammed in vivo. TGFβ production by malignant megakaryocytes plays a definitive role promoting myelofibrosis in MPNs. However, TGFβ was equally expressed by MSCs derived from MPLWT and MPLW515L expressing mice and the addition of neutralizing anti-TGFβ antibody only partially reduced collagen secretion in vitro. Interestingly, profibrotic MSCs displayed increased levels of pSmad3 and pSTAT3 suggesting that inflammatory mediators cooperating with the TGFβ-receptor signaling may maintain the aberrant phenotype ex vivo. FGFb is a known suppressor of TGFβ signaling. Reduced collagen deposition by FGFb-treated MSCs derived from MPLW515L mice suggests that the activating pathway is vulnerable to this suppressive mediator. Therefore, our findings have implications for the future investigation of therapies to reverse fibrosis in MPNs. PMID:28135282

Predictive factors for anemia response to erythropoiesis-stimulating agents in myelofibrosis.

PubMed

Hernández-Boluda, Juan-Carlos; Correa, Juan-Gonzalo; García-Delgado, Regina; Martínez-López, Joaquín; Alvarez-Larrán, Alberto; Fox, María-Laura; García-Gutiérrez, Valentín; Pérez-Encinas, Manuel; Ferrer-Marín, Francisca; Mata-Vázquez, María-Isabel; Raya, José-María; Estrada, Natalia; García, Silvia; Kerguelen, Ana; Durán, María-Antonia; Albors, Manuel; Cervantes, Francisco

2017-04-01

Erythropoiesis-stimulating agents (ESAs) are commonly used to treat the anemia of myelofibrosis (MF), but information on the predictors of response is limited. Results of ESA therapy were analyzed in 163 MF patients with severe anemia, most of whom had inadequate erythropoietin (EPO) levels (<125 U/L) at treatment start. According to the revised criteria of the International Working Group for Myelofibrosis Treatment and Research, anemia response was achieved in 86 patients (53%). Median response duration was 19.3 months. In multivariate analysis, baseline factors associated with a higher response rate were female sex (P=.007), leukocyte count ≥10×10 9 /L (P=.033), and serum ferritin <200 ng/mL (P=.002). Patients with 2 or 3 of the above features had a significantly higher response rate than the remainder (73% vs 28%, respectively; P<.001). Over the 373 patient-years of follow-up on ESA treatment, nine patients developed thrombotic complications (six arterial, three venous), accounting for 2.41 events per 100 patient-years. Survival time from ESA start was longer in anemia responders than in non-responders (P=.011). Besides the already established predictive value of EPO levels, these data can help to identify which MF patients are more likely to benefit from ESA treatment. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

The role of transforming growth factor-beta in PEG-rHuMGDF-induced reversible myelofibrosis in rats.

PubMed

Yanagida, M; Ide, Y; Imai, A; Toriyama, M; Aoki, T; Harada, K; Izumi, H; Uzumaki, H; Kusaka, M; Tokiwa, T

1997-12-01

Pegylated recombinant human megakaryocyte growth and development factor (PEG-rHuMGDF) injected at a suprapharmacologic dose (100 microg/kg) daily for 5 d in normal rats caused marked increases in marrow megakaryocytes and platelet counts at 6-8 d followed by gradual decreases to control levels at 10-20 d. Interestingly, in addition to the expected thrombopoiesis, PEG-rHuMGDF was associated with myelofibrosis with a predominance of reticulin fibres at day 10 followed by complete normalization by day 20. At 6-8 d, the levels of transforming growth factor-beta1 (TGF-beta1) in the extracellular fluid of the marrow, the platelet poor plasma, and the platelet extract were increased 23-, 7- and 2-fold, respectively. The elevated levels of TGF-beta1 were gradually reduced to baseline levels at 13-20 d in accordance with the normalization of myelofibrosis and thrombopoiesis. An ultrastructural analysis showed that large fragments of megakaryocytes were deposited in the marrow parenchyma of PEG-rHuMGDF-treated rats at day 6. PEG-rHuMGDF administration at pharmacologic doses (1 and 10 microg/kg) did not induce the deposition of reticulin fibres in the marrow. These findings suggest that TGF-beta1 leaked from megakaryocytes is involved in the development of the PEG-rHuMGDF-induced myelofibrosis and that this is a reversible process related to the regulation of the excess production of platelets.

Myelofibrosis associated with prominent periosteal bone apposition. Report of two cases.

PubMed

Yu, J S; Greenway, G; Resnick, D

1994-01-01

Myelofibrosis is a myeloproliferative disorder that is characterized by splenomegaly and bone marrow replacement by fibrous tissue. The predominant radiographic feature is osteosclerosis; however, in rare instances, periosteal bone apposition or periostitis is apparent in the metaphysis of the distal femura and proximal tibiae. It has been suggested that periostitis, when associated with fever and bone pain, is indicative of more aggressive disease. We report this unusual radiographic finding and its similar appearance to hypertrophic osteoarthropathy in two patients with myelofibrosis. In our patients, the presence of periosteal bone apposition did not correlate with increased disease aggressiveness.

Impact of Molecular Genetics on Outcome in Myelofibrosis Patients after Allogeneic Stem Cell Transplantation.

PubMed

Kröger, Nicolaus; Panagiota, Victoria; Badbaran, Anita; Zabelina, Tatjana; Triviai, Ioanna; Araujo Cruz, Michelle Maria; Shahswar, Rabia; Ayuk, Francis; Gehlhaar, Marten; Wolschke, Christine; Bollin, Robin; Walter, Carolin; Dugas, Martin; Wiehlmann, Lutz; Lehmann, Ulrich; Koenecke, Christian; Chaturvedi, Anuhar; Alchalby, Haefaa; Stadler, Michael; Eder, Matthias; Christopeit, Max; Göhring, Gudrun; Koenigsmann, Michael; Schlegelberger, Brigitte; Kreipe, Hans-Heinrich; Ganser, Arnold; Stocking, Carol; Fehse, Boris; Thol, Felicitas; Heuser, Michael

2017-07-01

Molecular genetics may influence outcome for patients with myelofibrosis. To determine the impact of molecular genetics on outcome after allogeneic stem cell transplantation, we screened 169 patients with primary myelofibrosis (n = 110), post-essential thrombocythemia/polycythemia vera myelofibrosis (n = 46), and myelofibrosis in transformation (n = 13) for mutations in 16 frequently mutated genes. The most frequent mutation was JAK2V617F (n = 101), followed by ASXL1 (n = 49), calreticulin (n = 34), SRSF2 (n = 16), TET2 (n = 10), U2AF1 (n = 11), EZH2 (n = 7), MPL (n = 6), IDH2 (n = 5), IDH1 (n = 4), and CBL (n = 1). The cumulative incidence of nonrelapse mortality (NRM) at 1 year was 21% and of relapse at 5 years 25%. The 5-year rates progression-free (PFS) and overall survival (OS) were and 56%, respectively. In a multivariate analysis CALR mutation was an independent factor for lower NRM (HR, .415; P = .05), improved PFS (HR, .393; P = .01), and OS (HR, .448; P = .03). ASXL1 and IDH2 mutations were independent risk factors for lower PFS (HR, 1.53 [P = .008], and HR, 5.451 [P = .002], respectively), whereas no impact was observed for "triple negative" patients. Molecular genetics, especially CALR, IDH2, and ASXL1 mutations, may thus be useful to predict outcome independently from known clinical risk factors after allogeneic stem cell transplantation for myelofibrosis. Copyright © 2017 The American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved.

High Frequency of Copy-Neutral Loss of Heterozygosity in Patients with Myelofibrosis.

PubMed

Rego de Paula Junior, Milton; Nonino, Alexandre; Minuncio Nascimento, Juliana; Bonadio, Raphael S; Pic-Taylor, Aline; de Oliveira, Silviene F; Wellerson Pereira, Rinaldo; do Couto Mascarenhas, Cintia; Forte Mazzeu, Juliana

2018-01-01

Myelofibrosis is the rarest and most severe type of Philadelphia-negative classical myeloproliferative neoplasms. Although mutually exclusive driver mutations in JAK2, MPL, or CALR that activate JAK-STAT pathway have been related to the pathogenesis of the disease, chromosome abnormalities have also been associated with the phenotype and prognosis of the disease. Here, we report the use of a chromosomal microarray platform consisting of both oligo and SNP probes to improve the detection of chromosome abnormalities in patients with myelofibrosis. Sixteen patients with myelofibrosis were tested, and the results were compared to karyotype analysis. Driver mutations in JAK2, MPL, or CALR were investigated by PCR and MLPA. Conventional cytogenetics revealed chromosome abnormalities in 3 out of 16 cases (18.7%), while chromosomal microarray analysis detected copy-number variations (CNV) or copy-neutral loss of heterozygosity (CN-LOH) alterations in 11 out of 16 (68.7%) patients. These included 43 CN-LOH, 14 deletions, 1 trisomy, and 1 duplication. Ten patients showed multiple chromosomal abnormalities, varying from 2 to 13 CNVs or CN-LOHs. Mutational status for JAK2, CALR, and MPL by MLPA revealed a total of 3/16 (18.7%) patients positive for the JAK2 V617F mutation, 9 with CALR deletion or insertion and 1 positive for MPL mutation. Considering that most of the CNVs identified were smaller than the karyotype resolution and the high frequency of CN-LOHs in our study, we propose that chromosomal microarray platforms that combine oligos and SNP should be used as a first-tier genetic test in patients with myelofibrosis. © 2018 S. Karger AG, Basel.

Cytogenetic abnormalities and their prognostic significance in idiopathic myelofibrosis: a study of 106 cases.

PubMed

Reilly, J T; Snowden, J A; Spearing, R L; Fitzgerald, P M; Jones, N; Watmore, A; Potter, A

1997-07-01

The prognostic significance of cytogenetic abnormalities was determined in 106 patients with well-characterized idiopathic myelofibrosis who were successfully karyotyped at diagnosis. 35% of the cases exhibited a clonal abnormality (37/106), whereas 65% (69/106) had a normal karyotype. Three characteristic defects, namely del(13q) (nine cases), del(20q) (eight cases) and partial trisomy 1q (seven cases), were present in 64.8% (24/37) of patients with clonal abnormalities. Kaplan-Meier plots and log rank analysis demonstrated an abnormal karyotype to be an adverse prognostic variable (P<0.001). Of the eight additional clinical and haematological parameters recorded at diagnosis, age (P<0.01), anaemia (haemoglobin < or = 10 g/dl: P<0.001), platelet (< or = 100 x 10(9)/l, P<0.0001) and leucocyte count (> 10.3 x 10(9)/l; P=0.06) were also associated with a shorter survival. In contrast, sex, spleen and liver size, and percentage blast cells were not found to be significant. Multivariate analysis, using Cox's regression, revealed karyotype, haemoglobin concentration, platelet and leucocyte counts to retain their unfavourable prognostic significance. A simple and useful schema for predicting survival in idiopathic myelofibrosis has been produced by combining age, haemoglobin concentration and karyotype with median survival times varying from 180 months (good-risk group) to 16 months (poor-risk group).

Sustained Erythroid Response in a Patient with Myelofibrosis Receiving Concomitant Treatment with Ruxolitinib and Deferasirox.

PubMed

Piro, Eugenio; Lentini, Maria; Levato, Luciano; Russo, Antonio; Molica, Stefano

2018-04-25

Iron overload (IOL) due to transfusion-dependent anemia is a serious adverse effect in patients with myelofibrosis (MF). Recent studies have shown that the oral iron chelator deferasirox may prevent multiple organ damage due to IOL in MF. However, it is not clear whether deferasirox may contribute to revert transfusion-dependent anemia. Here, we present a patient with transfusion-dependent intermediate-2 MF according to the International Prognostic Scoring System treated with ruxolitinib in combination with deferasirox. In addition to a reduced serum ferritin level, the patient required less blood transfusions, ultimately resulting in long-lasting transfusion-free survival. © 2018 S. Karger AG, Basel.

The Myelofibrosis Symptom Assessment Form (MFSAF): An Evidence-based Brief Inventory to Measure Quality of Life and Symptomatic Response to Treatment in Myelofibrosis

PubMed Central

Mesa, Ruben A.; Schwager, Susan; Radia, Deepti; Cheville, Andrea; Hussein, Kebede; Niblack, Joyce; Pardanani, Animesh D.; Steensma, David P.; Litzow, Mark R.; Rivera, Candido E.; Camoriano, John; Verstovsek, Srdan; Sloan, Jeffrey; Harrison, Claire; Kantarjian, Hagop; Tefferi, Ayalew

2015-01-01

Quality of life (QoL) in patients with myelofibrosis (MF) is severely compromised by severe constitutional symptoms (i.e. fatigue, night sweats, fever, weight loss), pruritus, and symptoms from frequently massive hepatosplenomegaly. Given that no current instrument of patient reported outcomes (PRO) exists that covers the unique spectrum of symptomatology seen in MF patients, we sought to develop a new PRO instrument for MF patients for use in therapeutic clinical trials. Utilizing data from an international internet based survey of 458 patients with MF we created a 20 item instrument (MFSAF: Myelofibrosis Symptom Assessment Form) which measures the symptoms reported by >10% of MF patients, and includes a measure of QoL. We subsequently validated the MFSAF in a prospective trial of MF patients involving patient and provider feedback, as well as comparison to other validated instruments used in cancer patients. The MFSAF results were highly correlated with other instruments, judged comprehensive and understandable by patients, and should be considered for evaluation of MF symptoms in therapeutic trials. PMID:19250674

A multinational, open-label, phase 2 study of ruxolitinib in Asian patients with myelofibrosis: Japanese subset analysis.

PubMed

Oritani, Kenji; Okamoto, Shinichiro; Tauchi, Tetsuzo; Saito, Shigeki; Ohishi, Kohshi; Handa, Hiroshi; Takenaka, Katsuto; Gopalakrishna, Prashanth; Amagasaki, Taro; Ito, Kazuo; Akashi, Koichi

2015-03-01

Ruxolitinib is a potent Janus kinase (JAK) 1/JAK2 inhibitor that has demonstrated rapid and durable improvements in splenomegaly and symptoms and a survival benefit in 2 phase 3 trials in patients with myelofibrosis. Ruxolitinib was well tolerated and effectively reduced splenomegaly and symptom burden in Asian patients with myelofibrosis in the Asian multinational, phase 2 Study A2202. We present a subset analysis of Japanese patients (n = 30) in Study A2202. At data cutoff, 22 patients were ongoing; 8 discontinued, mainly due to adverse events (n = 4). At week 24, 33 % of patients achieved ≥35 % reduction from baseline in spleen volume; 56.0 % achieved ≥50 % reduction from baseline in total symptom score, as measured by the 7-day Myelofibrosis Symptom Assessment Form v2.0. The most common adverse events were anemia (63 %), thrombocytopenia (40 %), nasopharyngitis (37 %), decreased platelet counts (30 %), and diarrhea (30 %). Dose reductions or interruptions due to hemoglobin decreases were more frequent in Japanese patients; no loss of efficacy and no discontinuations due to hematologic abnormalities were observed. Ruxolitinib was well tolerated in Japanese patients and provided substantial reductions in splenomegaly and myelofibrosis-related symptoms similar to those observed in the overall Asian population and phase 3 COMFORT studies.

Efficacy and Safety of Ruxolitinib in the Treatment of Patients with Myelofibrosis

PubMed Central

Yi, Cecilia Arana; Tam, Constantine S.; Verstovsek, Srdan

2016-01-01

The JAK 1 and JAK2 inhibitor ruxolitinib has approved indications in myelofibrosis, a BCR-ABL1-negative myeloproliferative neoplasm associated with progressive bone marrow fibrosis and shortened survival. In Phase III clinical studies, ruxolitinib provided rapid and durable improvement of myelofibrosis-related splenomegaly and symptoms irrespective of mutation status, and was associated with a survival advantage compared with placebo or best available therapy. Because of dose-dependent cytopenias, blood count monitoring and dose titration are important to optimize therapy. Specific precautions apply to the treatment of patients with or at risk of serious infections. Discontinuation of ruxolitinib generally leads to symptom return within 1 week. Ruxolitinib also is approved for treatment of patients with polycythemia vera who have had an inadequate response to or are intolerant of hydroxyurea. PMID:25757677

The Small Molecule Inhibitor G6 Significantly Reduces Bone Marrow Fibrosis and the Mutant Burden in a Mouse Model of Jak2-Mediated Myelofibrosis

PubMed Central

Kirabo, Annet; Park, Sung O.; Wamsley, Heather L.; Gali, Meghanath; Baskin, Rebekah; Reinhard, Mary K.; Zhao, Zhizhuang J.; Bisht, Kirpal S.; Keserű, György M.; Cogle, Christopher R.; Sayeski, Peter P.

2013-01-01

Philadelphia chromosome–negative myeloproliferative neoplasms, including polycythemia vera, essential thrombocytosis, and myelofibrosis, are disorders characterized by abnormal hematopoiesis. Among these myeloproliferative neoplasms, myelofibrosis has the most unfavorable prognosis. Furthermore, currently available therapies for myelofibrosis have little to no efficacy in the bone marrow and hence, are palliative. We recently developed a Janus kinase 2 (Jak2) small molecule inhibitor called G6 and found that it exhibits marked efficacy in a xenograft model of Jak2-V617F–mediated hyperplasia and a transgenic mouse model of Jak2-V617F–mediated polycythemia vera/essential thrombocytosis. However, its efficacy in Jak2-mediated myelofibrosis has not previously been examined. Here, we hypothesized that G6 would be efficacious in Jak2-V617F–mediated myelofibrosis. To test this, mice expressing the human Jak2-V617F cDNA under the control of the vav promoter were administered G6 or vehicle control solution, and efficacy was determined by measuring parameters within the peripheral blood, liver, spleen, and bone marrow. We found that G6 significantly reduced extramedullary hematopoiesis in the liver and splenomegaly. In the bone marrow, G6 significantly reduced pathogenic Jak/STAT signaling by 53%, megakaryocytic hyperplasia by 70%, and the Jak2 mutant burden by 68%. Furthermore, G6 significantly improved the myeloid to erythroid ratio and significantly reversed the myelofibrosis. Collectively, these results indicate that G6 is efficacious in Jak2-V617F–mediated myelofibrosis, and given its bone marrow efficacy, it may alter the natural history of this disease. PMID:22796437

Azacitidine and Sonidegib or Decitabine in Treating Patients With Myeloid Malignancies

ClinicalTrials.gov

2018-02-05

Chronic Myelomonocytic Leukemia; de Novo Myelodysplastic Syndrome; Essential Thrombocythemia; Myelodysplastic Syndrome; Myelodysplastic/Myeloproliferative Neoplasm; Polycythemia Vera; Previously Treated Myelodysplastic Syndrome; Primary Myelofibrosis; Recurrent Adult Acute Myeloid Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Untreated Adult Acute Myeloid Leukemia

Bone morphogenetic protein antagonist gene NOG is involved in myeloproliferative disease associated with myelofibrosis.

PubMed

Andrieux, Joris; Roche-Lestienne, Catherine; Geffroy, Sandrine; Desterke, Christophe; Grardel, Nathalie; Plantier, Isabelle; Selleslag, Dominik; Demory, Jean-Loup; Laï, Jean-Luc; Leleu, Xavier; Le Bousse-Kerdiles, Caroline; Vandenberghe, Peter

2007-10-01

In a case with secondary myelofibrosis occurring after essential thrombocythemia, cytogenetic analysis revealed an isolated translocation t(X;17)(q27;q22) in all cells. We found that a bacterial artificial chromosome (BAC) encompassing the breakpoint on chromosome 17 long arm contained only one gene, NOG. We therefore investigated the occurrence of this rare breakpoint in myeloproliferative disorders (MPDs). We identified three more patients with a 17q abnormality in MPDs: myelofibrosis with myeloid metaplasia (MMM); chronic myeloid leukemia positive for t(9;22)(q34;q11) with additional t(4;17)(p15;q22) at diagnosis; and myelofibrosis complicating polycythemia vera. All three cases exhibited a split of BACs containing NOG. The protein encoded by NOG, noggin, acts as an antagonist to bone morphogenetic secreted protein 2 and 4 (BMP2 and BMP4). A comparative analysis of gene expression on Agilent 22K oligonucleotide microarrays in purified CD34+ cells from the blood of MMM patients showed significant downregulation of BMPR2, BMPR1B, BMP2, and BMP8; upregulation of BMP3 and BMP10; and a trend to lower expression of NOG. Thus, given that expression and release of BMPs are important in the induction of osteosclerosis and angiogenic activity, the observed BMP deregulations could be triggered by potential NOG genetic alterations in the four cases here described, and may contribute to the myelofibrotic process characterized by bone marrow stromal reaction including collagen fibrosis, osteosclerosis, and angiogenesis.

The role of the extracellular matrix in primary myelofibrosis

PubMed Central

Leiva, O; Ng, S K; Chitalia, S; Balduini, A; Matsuura, S; Ravid, K

2017-01-01

Primary myelofibrosis (PMF) is a myeloproliferative neoplasm that arises from clonal proliferation of hematopoietic stem cells and leads to progressive bone marrow (BM) fibrosis. While cellular mutations involved in the development of PMF have been heavily investigated, noteworthy is the important role the extracellular matrix (ECM) plays in the progression of BM fibrosis. This review surveys ECM proteins contributors of PMF, and highlights how better understanding of the control of the ECM within the BM niche may lead to combined therapeutic options in PMF. PMID:28157219

Tardive dyskinesia successfully treated with alprazolam.

PubMed Central

Jordan, H. W.; Williams, B. C.

1990-01-01

Tardive dyskinesia is a disorder secondary to prolonged treatment (from 18 months to 3 years) with antipsychotic agents, affecting approximately 15% to 20% of patients. Tardive dyskinesia is characterized by difficulty controlling involuntary movements of the small muscle groups, producing tic-like reactions, muscle rigidity, and difficulty maintaining muscle tone. It is a chronic and unrelenting disorder which may be permanent if not successfully treated. The mechanism of action is thought to be secondary to dopamine hypersensitivity resulting from prolonged deprivation of dopamine on the part of dopamine-sensitive receptors. Theoretically, these receptors have been deprived of the neurotransmitter by chronic treatment with antipsychotic drugs, which are recognized as dopamine-blocking agents. We present a case in which alprazolam was successfully used in treating tardive dyskinesia. PMID:2213917

Regenerative nodular hyperplasia, portal vein thrombosis and primary myelofibrosis: an unusual triple association.

PubMed

Sández Montagut, Víctor Manuel; Giráldez Gallego, Álvaro; Ontanilla Clavijo, Guilermo

2018-03-01

We report a case of a regenerative nodular hyperplasia with a portal vein cavernomatosis with a subsequent progression to symptomatic, occlusive thrombosis of the superior mesenteric vein. A thorough investigation resulted in a final diagnosis of primary myelofibrosis associated with the V617F mutation in the JAK2 gene.

[Endodontically treated teeth. Success--failure. Endorestorative treatment plan].

PubMed

Zabalegui, B

1990-01-01

More and more often the general dentist is finding the presence of endodontically treated teeth during his treatment planning procedure. He has to ask himself if the endo-treated tooth functions and will continue to function function successfully, when deciding which final endo-restorative procedure to apply. For this reason the dentist or the endodontist with whom he works should clinically evaluate these teeth, establish a diagnostic criteria of their success or failure and a treatment plan according to the prognosis. The purpose of this article is to offer an organized clinical view of the steps to follow when evaluating an endodontically treated tooth and how to establish a final endo-restorative plan.

Inability of immunomorphometric assessment of angiogenesis to distinguish primary versus secondary myelofibrosis.

PubMed

Sharma, Prashant; Pati, Hara Prasad; Mishra, Pravas Chandra; Dinda, Amit Kumar; Gupta, Ruchika; Sharma, Alok; Jacob, Tony George

2011-08-01

To explore the utility of bone marrow (BM) angiogenesis in differentiating primary myelofibrosis (PMF) from secondary myelofibrosis (MF). CD34 immunostaining was performed on BM biopsies from 21 PMFs, 23 non-PMF myeloproliferative neoplasms (MPN) with associated MF, 20 secondary MF samples, and 10 nonfibrotic controls. Microvessel density (MVD) and microvessel surface area (MSA), along with blood and BM findings were compared between the groups. The post-MPN MF cases included chronic myeloid leukemia-MF and polycythemia vera-MF. Etiologies of secondary MF were metastatic carcinomas, non-MPN hematologic malignancies, tuberculosis, autoimmune MF, and osteopetrosis. Megakaryocytic clustering was the most frequent and intrasinusoidal hematopoiesis the most specific feature of PMF. Higher reticulin grade, collagenization, and osteomyelosclerosis were commoner in PMF. MVD and MSA were significantly increased in fibrotic marrows regardless of etiology. Although mean MVD as well as MSA were highest in PMF, extensive overlaps among groups and marked heterogeneity in the secondary MF group rendered them of limited utility in the differential diagnosis. Enhanced angiogenesis is not entirely specific for PMF. Overlaps with secondary MF limits its differential diagnostic utility. Pathogenetically, our findings suggest that enhanced angiogenesis is a secondary paraneoplastic stromal response shared by various unrelated conditions.

Fatal Disseminated Tuberculosis during Treatment with Ruxolitinib Plus Prednisolone in a Patient with Primary Myelofibrosis: A Case Report and Review of the Literature.

PubMed

Tsukamoto, Yasuhiro; Kiyasu, Junichi; Tsuda, Mariko; Ikeda, Motohiko; Shiratsuchi, Motoaki; Ogawa, Yoshihiro; Yufu, Yuji

2018-05-01

A 73-year-old man with primary myelofibrosis (PMF) was being treated with hydroxyurea, which was changed to ruxolitinib treatment because of worsening constitutional symptoms. Although ruxolitinib rapidly induced relief, he developed a high-grade fever. A comprehensive fever work-up found no apparent cause of the fever, except for PMF. Therefore, we increased the dose of ruxolitinib and added prednisolone, which was gradually withdrawn with resolution of the fever. However, the patient subsequently developed disseminated tuberculosis and died eight months after initiation of ruxolitinib. Our case highlights the importance of assessing and monitoring the immune status of patients receiving ruxolitinib.

Expanding the SHOC2 mutation associated phenotype of Noonan syndrome with loose anagen hair: structural brain anomalies and myelofibrosis.

PubMed

Gripp, Karen W; Zand, Dina J; Demmer, Laurie; Anderson, Carol E; Dobyns, William B; Zackai, Elaine H; Denenberg, Elizabeth; Jenny, Kim; Stabley, Deborah L; Sol-Church, Katia

2013-10-01

Noonan syndrome is a heterogenous rasopathy typically presenting with short stature, characteristic facial features, cardiac abnormalities including pulmonic valve stenosis, ASD and hypertrophic cardiomyopathy (HCM), cryptorchidism, ectodermal abnormalities, and learning differences. The phenotype is variable, and limited genotype phenotype correlation exists with SOS1 mutations often associated with normal cognition and stature, RAF1 mutations entailing a high HCM risk, and certain PTPN11 mutations predisposing to juvenile myelomonocytic leukemia. The recently identified SHOC2 mutation (p.Ser2Gly) causes Noonan syndrome with loose anagen hair. We report five patients with this mutation. All had skin hyperpigmentation, sparse light colored hair, increased fine wrinkles, ligamentous laxity, developmental delay, and 4/4 had a structural cardiac anomaly. Hypotonia and macrocephaly occurred in 4/5 (80%); 3/5 (60%) had polyhydramnios, increased birth weight or required use of a feeding tube. Distinctive brain abnormalities included relative megalencephaly and enlarged subarachnoid spaces suggestive of benign external hydrocephalus, and a relatively small posterior fossa as indicated by a vertical tentorium. The combination of a large brain with a small posterior fossa likely resulted in the high rate of cerebellar tonsillar ectopia (3/4; 75%). Periventricular nodular heterotopia was seen in one patient with a thick and dysplastic corpus callosum. We report on the first hematologic neoplasm, myelofibrosis, in a 2-year-old patient with SHOC2 mutation. Myelofibrosis is exceedingly rare in children and young adults. The absence of a somatic JAK2 mutation, seen in the majority of patients with myelofibrosis, is noteworthy as it suggests that germline or somatic SHOC2 mutations are causally involved in myelofibrosis. Copyright © 2013 Wiley Periodicals, Inc.

Cutaneous sarcoidosis successfully treated with alefacept.

PubMed

Garcia-Zuazaga, Jorge; Korman, Neil J

2006-01-01

Sarcoidosis is a systemic granulomatous disease of unknown etiology that affects multiple organ systems, including the pulmonary, lymphatic, skeletal, and integumentary systems. Improved understanding of the intrinsic immunology and molecular biology in sarcoidosis can be applied to the treatment of this disease. Alefacept is a human fusion protein consisting of the extracellular domain of leukocyte function-associated antigen 3 fused with the Fc portion of human immunoglobulin G1. It works by blocking the interaction between antigen-presenting cells and T cells to inhibit activation and by inducing apoptosis of CD4+ T cells. In this case report, we describe a 46-year-old patient with recalcitrant lupus pernio who was successfully treated with alefacept. To determine whether T-cell inhibition, specifically the use of alefacept, may be used to treat a patient with recalcitrant cutaneous sarcoidosis. Case report. There was a modest clinical improvement after 8 weeks of intramuscular injections of alefacept. This case report provides further evidence of successful treatment of sarcoidosis with biologic agents directed against T-lymphocyte activation.

Aggressive TAFRO syndrome with reversible cardiomyopathy successfully treated with combination chemotherapy.

PubMed

Yasuda, Shunichiro; Tanaka, Keisuke; Ichikawa, Ayako; Watanabe, Ken; Uchida, Emi; Yamamoto, Masahide; Yamamoto, Kouhei; Mizuchi, Daisuke; Miura, Osamu; Fukuda, Tetsuya

2016-10-01

TAFRO (thrombocytopenia, anasarca, myelofibrosis, renal dysfunction, and organomegaly) syndrome is an atypical manifestation of Castleman's disease. However, the mechanism underlying this very rare syndrome remains unknown, and there is no established standard treatment. Here we report cases of two young females with TAFRO syndrome who showed similar clinical courses. Both cases showed severe anasarca, ascites, and thrombocytopenia. Although high-dose steroids were ineffective, combination chemotherapy showed remarkable effects. However, both patients developed severe but reversible heart failure after CHOP therapy owing to diffuse cardiomyopathy, which was presumably associated with TAFRO syndrome. Therefore, although combination chemotherapy may be very effective in the treatment of TAFRO syndrome, careful observation for cardiomyopathy development is needed, particularly when using adriamycin-containing regimens.

[Myelofibrosis in a benzene-exposed cleaning worker].

PubMed

Bausà, Roser; Navarro, Lydia; Cortès-Franch, Imma

Long-term exposure to benzene has been associated with several blood malignancies, including aplastic anemia, myeloproliferative neoplasms, and different leukemias. We present a case of primary myelofibrosis in a 59-year-old woman who worked as a cleaner at a car dealership and automobile mechanic shop. For 25 years, she used gasoline as a degreaser and solvent to clean engine parts, floors and work desks on a daily basis. She was referred by her primary care provider to the Occupational Health Unit of Barcelona to assess whether her illness was work-related. Review of her job history and working conditions revealed chronic exposure to benzene in the absence of adequate preventive measures. An association between benzene exposure and myeloproliferative disease was established, suspicious for an occupational disease. Copyright belongs to the Societat Catalana de Salut Laboral.

Neutropenia caused by hairy cell leukemia in a patient with myelofibrosis secondary to polycythemia vera: a case report.

PubMed

Habberstad, Andreas Hanssønn; Tran, Hoa Thi Tuyet; Randen, Ulla; Spetalen, Signe; Dybedal, Ingunn; Tjønnfjord, Geir E; Dahm, Anders Erik Astrup

2018-04-24

Polycythemia vera is a myeloproliferative disease that sometimes evolves to myelofibrosis, causing splenomegaly and neutropenia. In this case report, we describe a patient with polycythemia vera and unexplained neutropenia who later turned out to also have hairy cell leukemia. A middle-aged Caucasian man with polycythemia vera presented to our hospital with chronic mouth ulcers. Later he developed leukopenia and pancytopenia. Bone marrow biopsies showed fibrosis. Further morphological analyses of bone marrow and blood smears revealed probable transformation into acute myeloid leukemia. However, there were also cells indicating hairy cell leukemia. Morphological and immunohistochemical analyses later confirmed the presence of hairy cell leukemia in biopsies that had been present for 3 years. Treatment with cladribine temporarily reversed the patient's neutropenia. Hairy cell leukemia may mimic development to myelofibrosis in patients with polycythemia vera.

A clinical-molecular prognostic model to predict survival in patients with post polycythemia vera and post essential thrombocythemia myelofibrosis.

PubMed

Passamonti, F; Giorgino, T; Mora, B; Guglielmelli, P; Rumi, E; Maffioli, M; Rambaldi, A; Caramella, M; Komrokji, R; Gotlib, J; Kiladjian, J J; Cervantes, F; Devos, T; Palandri, F; De Stefano, V; Ruggeri, M; Silver, R T; Benevolo, G; Albano, F; Caramazza, D; Merli, M; Pietra, D; Casalone, R; Rotunno, G; Barbui, T; Cazzola, M; Vannucchi, A M

2017-12-01

Polycythemia vera (PV) and essential thrombocythemia (ET) are myeloproliferative neoplasms with variable risk of evolution into post-PV and post-ET myelofibrosis, from now on referred to as secondary myelofibrosis (SMF). No specific tools have been defined for risk stratification in SMF. To develop a prognostic model for predicting survival, we studied 685 JAK2, CALR, and MPL annotated patients with SMF. Median survival of the whole cohort was 9.3 years (95% CI: 8-not reached-NR-). Through penalized Cox regressions we identified negative predictors of survival and according to beta risk coefficients we assigned 2 points to hemoglobin level <11 g/dl, to circulating blasts ⩾3%, and to CALR-unmutated genotype, 1 point to platelet count <150 × 10 9 /l and to constitutional symptoms, and 0.15 points to any year of age. Myelofibrosis Secondary to PV and ET-Prognostic Model (MYSEC-PM) allocated SMF patients into four risk categories with different survival (P<0.0001): low (median survival NR; 133 patients), intermediate-1 (9.3 years, 95% CI: 8.1-NR; 245 patients), intermediate-2 (4.4 years, 95% CI: 3.2-7.9; 126 patients), and high risk (2 years, 95% CI: 1.7-3.9; 75 patients). Finally, we found that the MYSEC-PM represents the most appropriate tool for SMF decision-making to be used in clinical and trial settings.

Indirect and non-medical economic burden, quality-of-life, and disabilities of the myelofibrosis disease in Spain.

PubMed

Gimenez, Emmanuel; Besses, Carles; Boque, Concepcion; Velez, Patricia; Kerguelen, Ana; Cervantes, Francisco; Ferrer-Marin, Francisca; Perez-Encinas, Manuel; Rodriguez, Mercedes; Gonzalez, Juan Diego; Calzada, Reyes; Hernandez-Boluda, Juan Carlos

2014-06-01

Myelofibrosis is a non-frequent chronic myeloproliferative Philadelphia-negative chromosome neoplasm. It is a heavy incapacitating orphan disease and associated with high morbidity and mortality. In this context, indirect and non-medical costs are expected to be high. The main objective of this project is to estimate the economic burden of this disease in Spain. Thirty-three patients with a diagnosis of myelofibrosis for at least 1 year participated in a questionnaire in three Spanish centers. The study consisted of analyzing in various aspects the cost and impact of the disease; indeed, daily life time limitations with a need of informal care, symtomatology. Additionally, information concerning the clinical management of the disease was collected through a focus group of eight experts. The mean age was 65 years. 15 of 33 patients were at their productive stage. Six had difficulties at work and eight have received informal care. Bone and muscular pain were the main symptoms of patients (72%). The estimated global indirect and non-medical costs of the disease were 86,315€ per patient (20% working and 80% informal care), which reached 104,153€ at productive stage patients (45%) and 168,459€ for more symptomatic patients. The economic burden of indirect and non-medical costs of myelofibrosis are important (15,142€/annual) as a result, and should be considered in economic evaluation, as well as in preventive plans for patients and caregivers, despite the fact that studies with larger numbers of patients should be done.

Splanchnic vein thrombosis as a first manifestation of Primary myelofibrosis

PubMed

Campos-Cabrera, Gregorio; Campos-Cabrera, Virginia; Campos-Cabrera, Salvador; Campos-Villagómez, José-Luis; Romero-González, Alejandra

2017-01-01

Myeloproliferative neoplasms are chronic disorders of clonal hematopoietic stem cells, characterized by an overproduction of functional granulocytes, red blood cells and / or platelets, and one of the major complications is the occurrence of venous and arterial thrombotic problems caused by increased platelet aggregation and thrombin generation. In this study 11 cases of primary myelofibrosis (PM) were evaluated and 2 debuted with splanchnic venous thrombosis (SVT); so after seeing the results of this study and of world literature, it is suggested that in patients with SVT, diagnostic methods for PM like the JAK2V617F mutation should be included. Copyright: © 2017 SecretarÍa de Salud

Persistent gastrointestinal bleeding successfully treated with aminocaproic acid

PubMed Central

Graham, D. R.; Walker, R. J.

1982-01-01

An elderly female presented with persistent upper gastrointestinal bleeding from a mucosal lesion in the second part of the duodenum. This failed to respond to surgery, but was successfully treated with aminocaproic acid. ImagesFig. 1Fig. 2 PMID:6983686

An accurate, simple prognostic model consisting of age, JAK2, CALR, and MPL mutation status for patients with primary myelofibrosis.

PubMed

Rozovski, Uri; Verstovsek, Srdan; Manshouri, Taghi; Dembitz, Vilma; Bozinovic, Ksenija; Newberry, Kate; Zhang, Ying; Bove, Joseph E; Pierce, Sherry; Kantarjian, Hagop; Estrov, Zeev

2017-01-01

In most patients with primary myelofibrosis, one of three mutually exclusive somatic mutations is detected. In approximately 60% of patients, the Janus kinase 2 gene is mutated, in 20%, the calreticulin gene is mutated, and in 5%, the myeloproliferative leukemia virus gene is mutated. Although patients with mutated calreticulin or myeloproliferative leukemia genes have a favorable outcome, and those with none of these mutations have an unfavorable outcome, prognostication based on mutation status is challenging due to the heterogeneous survival of patients with mutated Janus kinase 2. To develop a prognostic model based on mutation status, we screened primary myelofibrosis patients seen at the MD Anderson Cancer Center, Houston, USA, between 2000 and 2013 for the presence of Janus kinase 2, calreticulin, and myeloproliferative leukemia mutations. Of 344 primary myelofibrosis patients, Janus kinase 2 V617F was detected in 226 (66%), calreticulin mutation in 43 (12%), and myeloproliferative leukemia mutation in 16 (5%); 59 patients (17%) were triple-negatives. A 50% cut-off dichotomized Janus kinase 2-mutated patients into those with high Janus kinase 2 V617F allele burden and favorable survival and those with low Janus kinase 2 V617F allele burden and unfavorable survival. Patients with a favorable mutation status (high Janus kinase 2 V617F allele burden/myeloproliferative leukemia/calreticulin mutation) and aged 65 years or under had a median survival of 126 months. Patients with one risk factor (low Janus kinase 2 V617F allele burden/triple-negative or age >65 years) had an intermediate survival duration, and patients aged over 65 years with an adverse mutation status (low Janus kinase 2 V617F allele burden or triple-negative) had a median survival of only 35 months. Our simple and easily applied age- and mutation status-based scoring system accurately predicted the survival of patients with primary myelofibrosis. Copyright© Ferrata Storti Foundation.

Correction of anemia in a transfusion-dependent patient with primary myelofibrosis receiving iron chelation therapy with deferasirox (Exjade®, ICL670)

PubMed Central

Di Tucci, Anna Angela; Murru, Roberta; Alberti, Daniele; Rabault, Bertrand; Deplano, Simona; Angelucci, Emanuele

2007-01-01

Transfusional iron overload in patients with chronic anemias can result in multiple organ failure. Experience in the management of iron overload in patients with myelodysplastic syndromes is limited, as many do not receive chelation therapy due to short-life expectancy and the difficulties associated with the administration of the current reference standard chelator, deferoxamine. There have, however, been some reports of reduced transfusion requirement associated with chelation therapy in patients with myelodysplastic syndromes and myelofibrosis. Here, we discuss a patient with primary myelofibrosis and related transfusion-dependent anemia who received chelation therapy with the once-daily oral iron chelator, deferasirox. In addition to the reduced iron levels, the patient demonstrated an unexpected reduction in blood transfusion requirement, ultimately resulting in long-lasting transfusion-free survival. PMID:17391307

Broken Esophageal Stent Successfully Treated by Interventional Radiology Technique

DOE Office of Scientific and Technical Information (OSTI.GOV)

Zelenak, Kamil, E-mail: zelenak@mfn.s; Mistuna, Dusan; Lucan, Jaroslav

2010-06-15

Esophageal stent fractures occur quite rarely. A 61-year-old male patient was previously treated for rupture of benign stenosis, occurring after dilatation, by implanting an esophageal stent. However, a year after implantation, the patient suffered from dysphagia caused by the broken esophageal stent. He was treated with the interventional radiology technique, whereby a second implantation of the esophageal stent was carried out quite successfully.

Pacritinib versus best available therapy for the treatment of myelofibrosis irrespective of baseline cytopenias (PERSIST-1): an international, randomised, phase 3 trial.

PubMed

Mesa, Ruben A; Vannucchi, Alessandro M; Mead, Adam; Egyed, Miklos; Szoke, Anita; Suvorov, Aleksandr; Jakucs, Janos; Perkins, Andrew; Prasad, Ritam; Mayer, Jiri; Demeter, Judit; Ganly, Peter; Singer, Jack W; Zhou, Huafeng; Dean, James P; Te Boekhorst, Peter A; Nangalia, Jyoti; Kiladjian, Jean-Jacques; Harrison, Claire N

2017-05-01

Available therapies for myelofibrosis can exacerbate cytopenias and are not indicated for patients with severe thrombocytopenia. Pacritinib, which inhibits both JAK2 and FLT3, induced spleen responses with limited myelosuppression in phase 1/2 trials. We aimed to assess the efficacy and safety of pacritinib versus best available therapy in patients with myelofibrosis irrespective of baseline cytopenias. This international, multicentre, randomised, phase 3 trial (PERSIST-1) was done at 67 sites in 12 countries. Patients with higher-risk myelofibrosis (with no exclusions for baseline anaemia or thrombocytopenia) were randomly assigned (2:1) to receive oral pacritinib 400 mg once daily or best available therapy (BAT) excluding JAK2 inhibitors until disease progression or unacceptable toxicity. Randomisation was stratified by risk category, platelet count, and region. Treatment assignments were known to investigators, site personnel, patients, clinical monitors, and pharmacovigilance personnel. The primary endpoint was spleen volume reduction (SVR) of 35% or more from baseline to week 24 in the intention-to-treat population as assessed by blinded, centrally reviewed MRI or CT. We did safety analyses in all randomised patients who received either treatment. Here we present the final data. This trial is registered with ClinicalTrials.gov, number NCT01773187. Between Jan 8, 2013, and Aug 1, 2014, 327 patients were randomly assigned to pacritinib (n=220) or BAT (n=107). Median follow-up was 23·2 months (IQR 14·8-28·7). At week 24, the primary endpoint of SVR of 35% or more was achieved by 42 (19%) patients in the pacritinib group versus five (5%) patients in the BAT group (p=0·0003). 90 patients in the BAT group crossed over to receive pacritinib at a median of 6·3 months (IQR 5·8-6·7). The most common grade 3-4 adverse events through week 24 were anaemia (n=37 [17%]), thrombocytopenia (n=26 [12%]), and diarrhoea (n=11 [5%]) in the pacritinib group, and anaemia (n

Pharmacoeconomics of ruxolitinib therapy in patients with myelofibrosis.

PubMed

Vandewalle, Björn; Andreozzi, Valeska; Almeida, João; Félix, Jorge

2016-01-01

Overall survival (OS) and other important clinical trial end-points seem increasingly more elusive in supporting rapid and efficient incorporation of innovative cancer drugs in clinical practice. This study proposes a clinical trial based pharmacoeconomic framework to assess the therapeutic and economic value of ruxolitinib in patients with intermediate-2 or high-risk myelofibrosis. Individual patient level 144 week follow-up data from the COMFORT-II trial was used to account for the crossover effect on overall survival. Lifetime treatment benefits and costs were estimated considering detailed patterns of both ruxolitinib dose adjustments and blood transfusion needs. The authors estimate a 3.3 years increment in life expectancy (HR = 0.30; 95% CI = 0.17-0.55; p-value <0.001) and an incremental cost-effectiveness ratio of €40,000 per life year gained with the use of ruxolitinib. This study also demonstrates how valuable information from clinical trials can be used to support informed decisions about the early incorporation of innovative drugs.

A Patient with Localized Scleroderma Successfully Treated with Etretinate

PubMed Central

Shima, Tomoko; Yamamoto, Yuki; Ikeda, Takaharu; Furukawa, Fukumi

2014-01-01

There are several treatment methods for localized scleroderma, but treatment is difficult when the lesion is widely distributed. We encountered a case who was treated successfully with etretinate, a vitamin A derivative. The usefulness of this agent is discussed. PMID:25408646

Practical management of patients with myelofibrosis receiving ruxolitinib.

PubMed

Harrison, Claire; Mesa, Ruben; Ross, David; Mead, Adam; Keohane, Clodagh; Gotlib, Jason; Verstovsek, Srdan

2013-10-01

Myelofibrosis (MF) is characterized by bone marrow fibrosis, progressive anemia and extramedullary hematopoiesis, primarily manifested as splenomegaly. Patients also experience debilitating constitutional symptoms, including sequelae of splenomegaly, night sweats and fatigue. Ruxolitinib (INC424, INCB18424, Jakafi, Jakavi), a JAK1 and JAK2 inhibitor, was approved in November 2011 by the US FDA for the treatment of intermediate- or high-risk MF, and more recently in Europe and Canada for the treatment of MF-related splenomegaly or symptoms. These approvals were based on data from two randomized Phase III studies: COMFORT-I randomized against placebo, and COMFORT-II randomized against best available therapy. In these studies, ruxolitinib rapidly improved multiple disease manifestations of MF, reducing splenomegaly and improving quality of life of patients and potentially prolonging survival. However, as with other chemotherapies, ruxolitinib therapy is associated with some adverse events, such as anemia and thrombocytopenia. The aims of this article are to provide a brief overview of ruxolitinib therapy, to discuss some common adverse events associated with ruxolitinib therapy and to provide clinical management recommendations to maximize patients' benefit from ruxolitinib.

Idiopathic orbital inflammatory disease successfully treated with rituximab.

PubMed

Schafranski, Marcelo Derbli

2009-02-01

We report a case of a 66-year-old Caucasian female with a diagnosis of idiopathic orbital inflammatory disease (OID) refractory to azathioprine therapy. The coexistence of diabetes mellitus represented a relative contraindication to chronic prednisone use. After two infusions of rituximab, a chimeric anti-CD20+ antibody, ophthalmic signs and symptoms remarkably improved. To our knowledge, this is the first case of idiopathic OID successfully treated with rituximab.

Functional visual acuity in patients with successfully treated amblyopia: a pilot study.

PubMed

Hoshi, Sujin; Hiraoka, Takahiro; Kotsuka, Junko; Sato, Yumiko; Izumida, Shinya; Kato, Atsuko; Ueno, Yuta; Fukuda, Shinichi; Oshika, Tetsuro

2017-06-01

The aim of this study was to use conventional visual acuity measurements to quantify the functional visual acuity (FVA) in eyes with successfully treated amblyopia, and to compare the findings with those for contralateral normal eyes. Nineteen patients (7 boys, 12 girls; age 7.5 ± 2.2 years) with successfully treated unilateral amblyopia and the same conventional decimal visual acuity in both eyes (better than 1.0) were enrolled. FVA, the visual maintenance ratio (VMR), maximum and minimum visual acuity, and the average response time were recorded for both eyes of all patients using an FVA measurement system. The differences in FVA values between eyes were analyzed. The mean LogMAR FVA scores, VMR (p < 0.001 for both), and the LogMAR maximum (p < 0.005) and minimum visual acuity (p < 0.001) were significantly poorer for the eyes with treated amblyopia than for the contralateral normal eyes. There was no significant difference in the average response time. Our results indicate that FVA and VMR were poorer for eyes with treated amblyopia than for normal eyes, even though the treatment for amblyopia was considered successful on the basis of conventional visual acuity measurements. These results suggest that visual function is impaired in eyes with amblyopia, regardless of treatment success, and that FVA measurements can provide highly valuable diagnosis and treatment information that is not readily provided by conventional visual acuity measurements.

Successful treatment with tacrolimus in TAFRO syndrome: two case reports and literature review.

PubMed

Shirai, Taiichiro; Onishi, Akira; Waki, Daisuke; Saegusa, Jun; Morinobu, Akio

2018-06-01

TAFRO syndrome is a systemic inflammatory disorder characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly. In contrast to that in multicentric Castleman disease, interleukin-6 targeting strategies seem ineffective in some TAFRO syndrome cases; however, the optimal treatment remains unclear. Here, we report 2 cases of TAFRO syndrome, where 1 with cardiomyopathy, successfully treated with tacrolimus. This is the first case report of successful treatment with tacrolimus in TAFRO syndrome. Both patients (cases 1 and 2) developed fever, anasarca, thrombocytopenia, renal dysfunction, and mild hepatosplenomegaly. In both patients, lymph node pathology revealed mixed type Castleman disease-like features, and bone marrow showed reticulin myelofibrosis. TAFRO syndrome was diagnosed based on the patients' laboratory, clinical, and pathologic findings. In case 2, we observed a rare complication of cardiomyopathy with no evidence of takotsubo cardiomyopathy or viral myocarditis. In case 1, tocilizumab combined with glucocorticoids was ineffective and caused septic shock; additionally, cyclosporine A was discontinued because of hepatotoxicity. However, tacrolimus was effective in resolving TAFRO syndrome without any adverse events. In case 2, tacrolimus completely reversed TAFRO syndrome and was also effective in cardiomyopathy. This report suggests that tacrolimus is potentially effective and safe as an initial treatment and a glucocorticoid-sparing agent. Our literature review shows that calcineurin inhibitors, including tacrolimus, may be effective in TAFRO syndrome. Since previous studies indicate a role of Th1 inflammation in TAFRO syndrome pathogenesis, tacrolimus may, therefore, be effective in treating TAFRO syndrome.

Transfusion Independency and Histological Remission in a Patient with Advanced Primary Myelofibrosis Receiving Iron-Chelation Therapy with Deferasirox.

PubMed

Groepper, Stefanie; Schlue, Jerome; Haferlach, Claudia; Giagounidis, Aristoteles

2016-01-01

Iron overload is a common problem in patients with primary myelofibrosis and anemia due to transfusion dependency. This results in organ damage and toxic effects on hematopoietic cells in the bone marrow. At present, iron chelation therapy is not recommended in patients with myeloproliferative syndromes. We describe a very interesting development in a patient with primary myelofibrosis receiving iron chelation. Transfusion independency and a nearly complete histological remission of the underlying disease occurred within a few weeks of therapy. In addition, a change in molecular genetic findings was observed. Initially a JAK2 and a U2AF1 mutation were detected in the core biopsy. During and after therapy the U2AF1 mutation progressed, whereas the JAK2 mutation could no longer be verified. The improvement in hematopoiesis might results from reduction of oxidative stress on hematopoietic progenitor cells or other unclear deferasirox-mediated effects, whereas the reason for the change in molecular genetic findings is unclear. It appears that deferasirox might have a modulating effect on JAK2-kinase mutations. However, further investigation of selective molecular suppression properties of deferasirox are warranted. © 2016 S. Karger GmbH, Freiburg.

Efficacy of ALK5 inhibition in myelofibrosis

PubMed Central

Zhao, Wanke; Ho, Wanting Tina; Han, Ying; Murdun, Cem; Mailloux, Adam W.; Zhang, Ling; Wang, Xuefeng; Budhathoki, Anjali; Pradhan, Kith; Rapaport, Franck; Wang, Huaquan; Shao, Zonghong; Ren, Xiubao; Steidl, Ulrich; Levine, Ross L.; Zhao, Zhizhuang Joe; Verma, Amit; Epling-Burnette, Pearlie K.

2017-01-01

Myelofibrosis (MF) is a bone marrow disorder characterized by clonal myeloproliferation, aberrant cytokine production, extramedullary hematopoiesis, and bone marrow fibrosis. Although somatic mutations in JAK2, MPL, and CALR have been identified in the pathogenesis of these diseases, inhibitors of the Jak2 pathway have not demonstrated efficacy in ameliorating MF in patients. TGF-β family members are profibrotic cytokines and we observed significant TGF-β1 isoform overexpression in a large cohort of primary MF patient samples. Significant overexpression of TGF-β1 was also observed in murine clonal MPLW515L megakaryocytic cells. TGF-β1 stimulated the deposition of excessive collagen by mesenchymal stromal cells (MSCs) by activating the TGF-β receptor I kinase (ALK5)/Smad3 pathway. MSCs derived from MPLW515L mice demonstrated sustained overproduction of both collagen I and collagen III, effects that were abrogated by ALK5 inhibition in vitro and in vivo. Importantly, use of galunisertib, a clinically active ALK5 inhibitor, significantly improved MF in both MPLW515L and JAK2V617F mouse models. These data demonstrate the role of malignant hematopoietic stem cell (HSC)/TGF-β/MSC axis in the pathogenesis of MF, and provide a preclinical rationale for ALK5 blockade as a therapeutic strategy in MF. PMID:28405618

Toxic epidermal necrolysis successfully treated with etanercept.

PubMed

Gubinelli, Emanuela; Canzona, Flora; Tonanzi, Tiziano; Raskovic, Desanka; Didona, Biagio

2009-03-01

Toxic epidermal necrolysis (TEN) is a rare and acute severe adverse reaction to drugs, characterised by massive apoptosis and widespread epidermal and mucosal detachment. Although no gold standard therapy exists, human i.v. immunoglobulins have recently been described as an effective treatment for this disease. We report a case of phenobarbital-induced TEN in a 59-year-old white woman where the epidermal detachment stopped 48 h after beginning the etanercept treatment with complete healing after 20 days. To the best of our knowledge, this is only the second reported case of TEN successfully treated with etanercept.

Different expression patterns of LGALS1 and LGALS3 in polycythemia vera, essential thrombocythemia and primary myelofibrosis.

PubMed

Moura, L G; Tognon, R; Nunes, N S; Rodrigues, L Cataldi; Ferreira, A F; Kashima, S; Covas, D T; Santana, M; Souto, E X; Perobelli, L; Simões, B P; Dias-Baruffi, M; Castro, F A

2016-10-01

Despite all the knowledge, the cellular and molecular mechanisms involved in myeloproliferative neoplasm (MPN) pathophysiology remain unclear. Authors have shown galectin-1 (Gal-1) and 3 playing roles in tumour angiogenesis and fibrosis, which were correlated with poor prognosis in patients with MPN. In the present study LGALS1 and LGALS3 were differently expressed between polycythemia vera, essential thrombocythemia (ET) and primary myelofibrosis (PMF) diseases. Increased LGALS3 expression was associated with a negative JAK2 V617F status mutation in leucocytes from PMF but not in patients with ET without this mutation. However, a positive Janus kinase 2 (JAK2) V617F cell line established from patients with ET (SET-2 cells) when treated with JAK inhibitor presented high levels of LGALS3. Additionally, high LGALS1 expression was found in CD34(+) cells but not in leucocytes from patients with PMF, in absence of JAK2 V617F mutation, and also in SET-2 cells treated with JAK inhibitor. Thus, our findings indicate that differential expression of LGALS1 and/or LGALS3 in patients with MPN is linked with JAK2 V617F status mutation in these diseases and state of cell differentiation. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

Crohn's disease complicated by Epstein-Barr virus-driven haemophagocytic lymphohistiocytosis successfully treated with rituximab.

PubMed

Thompson, Grace; Pepperell, Dominic; Lawrence, Ian; McGettigan, Benjamin David

2017-02-22

We report a case of Epstein-Barr virus (EBV)-driven haemophagocytic lymphohistiocytosis (HLH) in a man with Crohn's disease treated with 6-mercaptopurine and adalimumab therapy who was successfully treated with rituximab therapy alone. This is the first published case in an adult patient with EBV-driven HLH in the setting of thiopurine use and inflammatory bowel disease to be successfully treated with rituximab therapy alone. Here, we will discuss putative immunological mechanisms which may contribute to this potentially life-threatening complication. 2017 BMJ Publishing Group Ltd.

The Amelioration of Myelofibrosis with Thrombocytopenia by a JAK1/2 Inhibitor, Ruxolitinib, in a Post-polycythemia Vera Myelofibrosis Patient with a JAK2 Exon 12 Mutation.

PubMed

Ikeda, Kazuhiko; Ueda, Koki; Sano, Takahiro; Ogawa, Kazuei; Ikezoe, Takayuki; Hashimoto, Yuko; Morishita, Soji; Komatsu, Norio; Ohto, Hitoshi; Takeishi, Yasuchika

2017-01-01

Less than 5% of patients with polycythemia vera (PV) show JAK2 exon 12 mutations. Although PV patients with JAK2 exon 12 mutations are known to develop post-PV myelofibrosis (MF) as well as PV with JAK2V617F, the role of JAK inhibitors in post-PV MF patients with JAK2 exon 12 mutations remains unknown. We describe how treatment with a JAK1/2 inhibitor, ruxolitinib, led to the rapid amelioration of marrow fibrosis, erythrocytosis and thrombocytopenia in a 77-year-old man with post-PV MF who carried a JAK2 exon 12 mutation (JAK2H538QK539L). This case suggests that ruxolitinib is a treatment option for post-PV MF in patients with thrombocytopenia or JAK2 exon 12 mutations.

Autoimmune myelofibrosis with pancytopenia as a presenting manifestation of systemic lupus erythematosus responsive to mycophenolate mofetil.

PubMed

Ungprasert, P; Chowdhary, V R; Davis, M D; Makol, A

2016-04-01

Hematological abnormalities, such as anemia, leucopenia, and thrombocytopenia, secondary to peripheral destruction, are common in systemic lupus erythematosus (SLE). However, cytopenias from autoimmune myelofibrosis (AIMF) are extremely uncommon in SLE, with less than 40 reported cases in the literature. We report the case of a 33-year-old female who presented with bullous skin lesions and pancytopenia as the presenting manifestation of what was ultimately diagnosed as SLE with AIMF. She responded well to glucocorticoids and mycophenolate mofetil. © The Author(s) 2015.

Orbital recurrence of retinoblastoma successfully treated by combined therapy.

PubMed Central

Goble, R R; McKenzie, J; Kingston, J E; Plowman, P N; Hungerford, J L

1990-01-01

Five children with an orbital recurrence of retinoblastoma have been successfully treated by a combination of excision biopsy of the tumour mass, radical orbital radiotherapy, and systemic chemotherapy. Nine previous children, consecutive with the five presented here, died from disseminated retinoblastoma after failure of earlier treatment programmes for orbital recurrence. An aggressive therapeutic approach is justified by this improvement in survival. PMID:2310733

Calreticulin mutants in mice induce an MPL-dependent thrombocytosis with frequent progression to myelofibrosis.

PubMed

Marty, Caroline; Pecquet, Christian; Nivarthi, Harini; El-Khoury, Mira; Chachoua, Ilyas; Tulliez, Micheline; Villeval, Jean-Luc; Raslova, Hana; Kralovics, Robert; Constantinescu, Stefan N; Plo, Isabelle; Vainchenker, William

2016-03-10

Frameshift mutations in the calreticulin (CALR) gene are seen in about 30% of essential thrombocythemia and myelofibrosis patients. To address the contribution of the CALR mutants to the pathogenesis of myeloproliferative neoplasms, we engrafted lethally irradiated recipient mice with bone marrow cells transduced with retroviruses expressing these mutants. In contrast to wild-type CALR, CALRdel52 (type I) and, to a lesser extent, CALRins5 (type II) induced thrombocytosis due to a megakaryocyte (MK) hyperplasia. Disease was transplantable into secondary recipients. After 6 months, CALRdel52-, in contrast to rare CALRins5-, transduced mice developed a myelofibrosis associated with a splenomegaly and a marked osteosclerosis. Monitoring of virus-transduced populations indicated that CALRdel52 leads to expansion at earlier stages of hematopoiesis than CALRins5. However, both mutants still specifically amplified the MK lineage and platelet production. Moreover, a mutant deleted of the entire exon 9 (CALRdelex9) did not induce a disease, suggesting that the oncogenic property of CALR mutants was related to the new C-terminus peptide. To understand how the CALR mutants target the MK lineage, we used a cell-line model and demonstrated that the CALR mutants, but not CALRdelex9, specifically activate the thrombopoietin (TPO) receptor (MPL) to induce constitutive activation of Janus kinase 2 and signal transducer and activator of transcription 5/3/1. We confirmed in c-mpl- and tpo-deficient mice that expression of Mpl, but not of Tpo, was essential for the CALR mutants to induce thrombocytosis in vivo, although Tpo contributes to disease penetrance. Thus, CALR mutants are sufficient to induce thrombocytosis through MPL activation. © 2016 by The American Society of Hematology.

F-18-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Appearance of Extramedullary Hematopoesis in a Case of Primary Myelofibrosis

PubMed Central

Mukherjee, Anirban; Bal, Chandrasekhar; Tripathi, Madhavi; Das, Chandan Jyoti; Shamim, Shamim Ahmed

2017-01-01

A 44-year-old female with known primary myelofibrosis presented with shortness of breath. High Resolution Computed Tomography thorax revealed large heterogeneously enhancing extraparenchymal soft tissue density mass involving bilateral lung fields. F-18-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography revealed mildly FDG avid soft tissue density mass with specks of calcification involving bilateral lung fields, liver, and spleen. Subsequent histopathologic evaluation from the right lung mass was suggestive of extramedullary hematopoesis. PMID:28533647

Successful treatment with tacrolimus in TAFRO syndrome: two case reports and literature review

PubMed Central

Shirai, Taiichiro; Onishi, Akira; Waki, Daisuke; Saegusa, Jun; Morinobu, Akio

2018-01-01

Abstract Rationale: TAFRO syndrome is a systemic inflammatory disorder characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly. In contrast to that in multicentric Castleman disease, interleukin-6 targeting strategies seem ineffective in some TAFRO syndrome cases; however, the optimal treatment remains unclear. Here, we report 2 cases of TAFRO syndrome, where 1 with cardiomyopathy, successfully treated with tacrolimus. This is the first case report of successful treatment with tacrolimus in TAFRO syndrome. Patient concerns: Both patients (cases 1 and 2) developed fever, anasarca, thrombocytopenia, renal dysfunction, and mild hepatosplenomegaly. Diagnoses: In both patients, lymph node pathology revealed mixed type Castleman disease-like features, and bone marrow showed reticulin myelofibrosis. TAFRO syndrome was diagnosed based on the patients’ laboratory, clinical, and pathologic findings. In case 2, we observed a rare complication of cardiomyopathy with no evidence of takotsubo cardiomyopathy or viral myocarditis. Interventions and outcomes: In case 1, tocilizumab combined with glucocorticoids was ineffective and caused septic shock; additionally, cyclosporine A was discontinued because of hepatotoxicity. However, tacrolimus was effective in resolving TAFRO syndrome without any adverse events. In case 2, tacrolimus completely reversed TAFRO syndrome and was also effective in cardiomyopathy. Lessons: This report suggests that tacrolimus is potentially effective and safe as an initial treatment and a glucocorticoid-sparing agent. Our literature review shows that calcineurin inhibitors, including tacrolimus, may be effective in TAFRO syndrome. Since previous studies indicate a role of Th1 inflammation in TAFRO syndrome pathogenesis, tacrolimus may, therefore, be effective in treating TAFRO syndrome. PMID:29879072

Warfarin-induced calciphylaxis successfully treated with sodium thiosulphate.

PubMed

Hafiji, Juber; Deegan, Patrick; Brais, Rebecca; Norris, Paul

2013-05-01

Calciphylaxis is a rare life-threatening form of skin necrosis. Although traditionally observed in patients with end-stage renal disease and/or hyperparathyroidism, calciphylaxis has also been reported to occur in 'non-traditional' patients with normal renal and parathyroid function. We report a case of warfarin-induced calciphylaxis treated successfully with sodium thiosulphate and discuss the role of Vitamin K2 as a potential therapeutic option in the management of warfarin-induced calciphylaxis. © 2012 The Authors. Australasian Journal of Dermatology © 2012 The Australasian College of Dermatologists.

Chronic idiopathic myelofibrosis terminating in extramedullary anaplastic plasmacytoma.

PubMed

Liu, Min-Ling; Kallakury, Bhaskar; Kessler, Craig; Hartmann, Dan-Paul; Azumi, Norio; Ozdemirli, Metin

2006-02-01

Chronic idiopathic myelofibrosis (CIMF) is a chronic myeloproliferative disorder (CMPD) with progressive fibrosis and extramedullary hematopoiesis. Similar to other CMPDs, the stem cell in CIMF has the potential to differentiate into myeloid or lymphoid lineages, and thus CIMF can culminate in acute leukemia of myeloid or, rarely, lymphoid lineage. We describe an unusual case of CIMF terminating in extramedullary anaplastic plasmacytoma. The patient was a 61-year-old male with an 11-year history of CIMF. His course was complicated by rapidly growing abdominal and inguinal lymphadenopathy. Lymph node biopsy revealed a diffuse undifferentiated infiltrate in the background of extramedullary hematopoiesis. Flow cytometric and immunohistochemical analysis demonstrated plasma cell-related antigens (CD138, CD38, cytoplasmic kappa light chain), epithelial membrane antigen and CD43 in the tumor cells. The myeloid, B-cell or T-cell markers were negative. A clonal immunoglobulin heavy chain gene rearrangement was identified by polymerase chain reaction. The plasma cell origin was further confirmed by electron microscopic examination, which revealed stacks of rough endoplasmic reticulum. Monoclonal gammopathy may occur in CIMF, and rare cases of simultaneous plasma cell myeloma and CIMF have been reported in the literature. However, to the best of our knowledge, this is the first report of CIMF terminating in extramedullary anaplastic plasmacytoma.

Clinical utility of routine MPL exon 10 analysis in the diagnosis of essential thrombocythaemia and primary myelofibrosis.

PubMed

Boyd, Elaine M; Bench, Anthony J; Goday-Fernández, Andrea; Anand, Shubha; Vaghela, Krishna J; Beer, Phillip; Scott, Mike A; Bareford, David; Green, Anthony R; Huntly, Brian; Erber, Wendy N

2010-04-01

Approximately 50% of essential thrombocythaemia and primary myelo-fibrosis patients do not have a JAK2 V617F mutation. Up to 5% of these are reported to have a MPL exon 10 mutation but testing for MPL is not routine as there are multiple mutation types. The ability to routinely assess both JAK2 and MPL mutations would be beneficial in the differential diagnosis of unexplained thrombocytosis or myelofibrosis. We developed and applied a high resolution melt (HRM) assay, capable of detecting all known MPL mutations in a single analysis, for the detection of MPL exon 10 mutations. We assessed 175 ET and PMF patients, including 67 that were JAK2 V617F-negative by real time polymerase chain reaction (PCR). Overall, 19/175 (11%) patients had a MPL exon 10 mutation, of whom 16 were JAK2 V617F-negative (16/67; 24%). MPL mutation types were W515L (11), W515K (4), W515R (2) and W515A (1). One patient had both W515L and S505N MPL mutations and these were present in the same haemopoietic colonies. Real time PCR for JAK2 V617F analysis and HRM for MPL exon 10 status identified one or more clonal marker in 71% of patients. This combined genetic approach increases the sensitivity of meeting the World Health Organization diagnostic criteria for these myeloproliferative neoplasms.

Treatment and management of myelofibrosis in the era of JAK inhibitors

PubMed Central

Keohane, Clodagh; Radia, Deepti H; Harrison, Claire N

2013-01-01

Myelofibrosis (MF) can present as a primary disorder or evolve from polycythemia vera (PV) or essential thrombocythemia (ET) to post-PV MF or post-ET MF, respectively. MF is characterized by bone marrow fibrosis, splenomegaly, leukoerythroblastosis, extramedullary hematopoiesis, and a collection of debilitating symptoms. Until recently, the therapeutic options for patients with MF consisted of allogeneic hematopoietic stem cell transplant (alloHSCT), the use of cytoreductive agents (ie, hydroxyurea), splenectomy and splenic irradiation for treatment of splenomegaly, and management of anemia with transfusions, erythropoiesis-stimulating agents (ESAs), androgens, and immunomodulatory agents. However, with increased understanding of the pathogenesis of MF resulting from dysregulated Janus kinase (JAK) signaling, new targeted JAK inhibitor therapies, such as ruxolitinib, are now available. The purpose of this article is to review the clinical features of MF, discuss the use and future of JAK inhibitors, reassess when and how to use conventional MF treatments in the context of JAK inhibitors, and provide a perspective on the future of MF treatment. PMID:23990704

Treatment and management of myelofibrosis in the era of JAK inhibitors.

PubMed

Keohane, Clodagh; Radia, Deepti H; Harrison, Claire N

2013-01-01

Myelofibrosis (MF) can present as a primary disorder or evolve from polycythemia vera (PV) or essential thrombocythemia (ET) to post-PV MF or post-ET MF, respectively. MF is characterized by bone marrow fibrosis, splenomegaly, leukoerythroblastosis, extramedullary hematopoiesis, and a collection of debilitating symptoms. Until recently, the therapeutic options for patients with MF consisted of allogeneic hematopoietic stem cell transplant (alloHSCT), the use of cytoreductive agents (ie, hydroxyurea), splenectomy and splenic irradiation for treatment of splenomegaly, and management of anemia with transfusions, erythropoiesis-stimulating agents (ESAs), androgens, and immunomodulatory agents. However, with increased understanding of the pathogenesis of MF resulting from dysregulated Janus kinase (JAK) signaling, new targeted JAK inhibitor therapies, such as ruxolitinib, are now available. The purpose of this article is to review the clinical features of MF, discuss the use and future of JAK inhibitors, reassess when and how to use conventional MF treatments in the context of JAK inhibitors, and provide a perspective on the future of MF treatment.

Epidemiology of myelofibrosis, essential thrombocythemia, and polycythemia vera in the European Union.

PubMed

Moulard, Odile; Mehta, Jyotsna; Fryzek, Jon; Olivares, Robert; Iqbal, Usman; Mesa, Ruben A

2014-04-01

Primary myelofibrosis (PMF), essential thrombocythemia (ET), and polycythemia vera (PV) are BCR ABL-negative myeloproliferative neoplasms (MPN). Published epidemiology data are scarce, and multiple sources are needed to assess the disease burden. We assembled the most recent information available on the incidence and prevalence of myelofibrosis (MF), ET, and PV by conducting a structured and exhaustive literature review of the published peer-reviewed literature in EMBASE and by reviewing online documentation from disease registries and relevant health registries in European countries. The search was restricted to human studies written in English or French and published between January 1, 2000, and December 6, 2012. Eleven articles identified from EMBASE, three online hematology or oncology registries, and two Web-based databases or reports were used to summarize epidemiological estimates for MF, PV, and ET. The incidence rate of MF ranged from 0.1 per 100,000 per year to 1 per 100,000 per year. Among the various registries, the incidence of PV ranged from 0.4 per 100,000 per year to 2.8 per 100,000 per year, while the literature estimated the range of PV incidence to be 0.68 per 100,000 to 2.6 per 100,000 per year. The estimated incidence of ET was between 0.38 per 100,000 per year and 1.7 per 100,000 per year. While a few studies reported on the MPNs' prevalences, it is difficult to compare them as various types of prevalence were calculated (point prevalence vs. period prevalence) and standardization was made according to different populations (e.g., the world population and the European population). There is a wide variation in both prevalence and incidence estimates observed across European data sources. Carefully designed studies, with standardized definitions of MPNs and complete ascertainment of patients including both primary and secondary MFs, should be conducted so that estimates of the population aimed to receive novel treatments for these neoplasms are

Autoimmune myelofibrosis accompanied by Sjögren's syndrome in a 47, XXX/46, XX mosaic woman.

PubMed

Takahashi, Tohru

2014-01-01

This report describes a patient with autoimmune myelofibrosis accompanied by Sjögren's syndrome (SS). A 36-year-old woman was admitted due to petechiae, purpura, gingival bleeding, dyspnea on exertion, and a lack of concentration. She had pancytopenia and was diagnosed with SS. A bone marrow study showed hypercellular marrow with reticulin fibrosis. Lymphocytic infiltrates and aggregates composed of a mixture of T and B cells in the marrow were also observed. A chromosomal analysis of the marrow cells showed 47, XXX and an analysis of peripheral lymphocytes revealed 47, XXX/46, XX mosaic results. The patient's cytopenia resolved following treatment with oral prednisolone.

Aspergillus flavus mycetoma and epidural abscess successfully treated with itraconazole.

PubMed

Witzig, R S; Greer, D L; Hyslop, N E

1996-01-01

Aspergillus spp. rarely cause mycetomata. We report a patient with diabetes and nephrotic syndrome with Aspergillus flavus mycetoma of the back, with the development of an epidural abscess, diskitis and vertebral osteomyelitis. The patient was successfully treated with decompressive laminectomy and a 14-month itraconazole regimen. Serial serum itraconazole levels and quantitative Aspergillus antigen levels were performed. This is the second reported and first extrapedal case of mycetoma caused by A. flavus.

A unique and unexplained ricochet leak post PCI - successfully treated with intra-coronary glue.

PubMed

Goel, Pravin K; Syal, Sanjeev K

2014-01-01

We herein describe a unique case of coronary artery perforation treated with covered stent with repeat cardiac tamponade resulting out of a fresh unexplained leak from a remote vessel (Ricochet) and successfully treated with intra-coronary injection of sterile synthetic glue, cyanoacrylate. Copyright © 2014 Cardiological Society of India. Published by Elsevier B.V. All rights reserved.

Ruptured renal arteriovenous malformation successfully treated by catheter embolization: a case report.

PubMed

Takeuchi, Nobuhiro; Nomura, Yusuke

2014-01-09

Renal arteriovenous fistula (RAVF) is a comparatively rare malformation. Here, we report a case of ruptured RAVF that was successfully treated by catheter embolization. An 89-year-old female was transferred to our institution with massive gross hematuria in March 2011. Plain abdominal computed tomography (CT) revealed dilated left renal pelvis with high-density contents. Hematoma was suspected. Subsequent plain abdominal magnetic resonance imaging revealed left hydronephrosis and blood retention in the dilated left renal pelvis. No renal or ureteral cancer was evident. Hematuria was conservatively treated using hemostatic agents but hematuria persisted. Repeated urinary cytology revealed no malignant cells. On day 9, the patient went into septic and/or hemorrhagic shock. Fluid and catecholamine infusion, blood transfusion, and antibacterial drugs were rapidly initiated, and the patient's general condition gradually improved. Contrast-enhanced abdominal CT revealed marked expansion of the hematoma in the renal pelvis and microaneurysms in the segmental arteries of the left kidney. Inflammation improved, and a left double-J stent was inserted. Selective renal angiography revealed RAVF with microaneurysms in the left segmental arteries; therefore, catheter embolization using metallic coils was performed, which resolved hematuria. We report a case of ruptured renal arteriovenous malformation, which was successfully treated by catheter embolization.

Catatonia after deep brain stimulation successfully treated with lorazepam and right unilateral electroconvulsive therapy: a case report.

PubMed

Quinn, Davin K; Rees, Caleb; Brodsky, Aaron; Deligtisch, Amanda; Evans, Daniel; Khafaja, Mohamad; Abbott, Christopher C

2014-09-01

The presence of a deep brain stimulator (DBS) in a patient who develops neuropsychiatric symptoms poses unique diagnostic challenges and questions for the treating psychiatrist. Catatonia has been described only once, during DBS implantation, but has not been reported in a successfully implanted DBS patient. We present a case of a patient with bipolar disorder and renal transplant who developed catatonia after DBS for essential tremor. The patient was successfully treated for catatonia with lorazepam and electroconvulsive therapy after careful diagnostic workup. Electroconvulsive therapy has been successfully used with DBS in a handful of cases, and certain precautions may help reduce potential risk. Catatonia is a rare occurrence after DBS but when present may be safely treated with standard therapies such as lorazepam and electroconvulsive therapy.

A Review of Ruxolitinib for the Treatment of Myelofibrosis: A Critique of the Evidence.

PubMed

Wade, Ros; Hodgson, Robert; Biswas, Mousumi; Harden, Melissa; Woolacott, Nerys

2017-02-01

As part of the National Institute for Health and Care Excellence's (NICE) Single Technology Appraisal (STA) process, ruxolitinib was assessed to determine the clinical and cost effectiveness of its use in the treatment of disease-related splenomegaly or symptoms in adults with myelofibrosis. Ruxolitinib had previously been assessed as part of the STA process and was not recommended in NICE guidance issued in June 2013 (TA289). A review of TA289 was commissioned following the availability of new longer-term survival data; a price discount patient access scheme (PAS) was also introduced. The Centre for Reviews and Dissemination (CRD) and Centre for Health Economics (CHE) Technology Appraisal Group at the University of York was commissioned to act as the independent Evidence Review Group (ERG). This article provides a summary of the manufacturer or sponsor of the technology's (referred to as the company) submission, the ERG review and the resulting NICE guidance issued in March 2016. The main clinical effectiveness data were derived from two good-quality multicentre randomised controlled trials (RCTs): COMFORT-II compared ruxolitinib with best available therapy (BAT) and COMFORT-I compared ruxolitinib with placebo. Both RCTs demonstrated a statistically significant reduction in splenomegaly and its associated symptoms in intermediate-2 and high-risk myelofibrosis patients. Overall survival was statistically significantly improved with ruxolitinib compared with BAT at 3.5 years of follow-up in the COMFORT-II trial (hazard ratio 0.58, 95 % CI 0.36-0.93). Grade 3-4 adverse events were more frequent in the ruxolitinib group than in the BAT group; 42 % compared with 25 %. Evidence relating to patients with lower-risk disease or low platelet counts (50-100 × 10 9 /L) was less robust. The company's economic model was well-presented and had an appropriate model structure. The base-case incremental cost-effectiveness ratio (ICER) was estimated to be around £45,000 per

Chronic total occlusion successfully treated with a bioresorbable everolimus-eluting vascular scaffold

PubMed Central

Mattesini, Alessio; Dall'Ara, Gianni; Mario, Carlo Di

2014-01-01

Fully bioresorbable vascular scaffolds (BVS) are a new approach to the percutaneous treatment of coronary artery disease. The BVS have not yet been fully tested in complex lesions, including chronic total occlusion (CTO). We report a CTO case successfully treated with a second-generation bioabsorbable drug-eluting scaffold. PMID:25061461

Factors predicting recurrence in successfully treated cases of anisometropic amblyopia.

PubMed

Saxena, Rohit; Puranik, Shraddha; Singh, Digvijay; Menon, Vimla; Sharma, Pradeep; Phuljhele, Swati

2013-11-01

Recurrence after successful treatment of amblyopia is known and understanding the risk factors could help effective management. To measure incidence of recurrence in successfully treated cases of anisometropic amblyopia and evaluate factors predicting it. Cohort Study at a tertiary level institution. Successfully treated anisometropic amblyopes aged 4-12 years were followed up for 1 year after stopping therapy. Best corrected visual acuity (BCVA), refractive error, stereoacuity and contrast sensitivity were evaluated at baseline and follow-up. Intergroup analysis with appropriate tests: Chi-square test, Fisher's exact test, Wilcoxon rank sum test and paired t-test. One hundred and two patients with mean age at diagnosis 7.06 μ 1.81 years were followed-up for a mean duration of 1.0 μ 0.2 years. The mean pre-treatment BCVA (LogMAR score) at diagnosis was 0.73 μ 0.36 units which improved to 0.20 μ 0.00 with treatment and after 1 year of stopping treatment was 0.22 μ 0.07. Thirteen (12.74%) patients showed amblyopia recurrence during follow-up. Risk of recurrence was higher with older age of onset of treatment (6.64 μ 1.77 years without recurrence v/s 8.53 μ 1.39 years with recurrence, P = 0.0014). Greater extent of improvement of VA (P = 0.048) and final VA at stopping occlusion (P = 0.03) were associated with higher recurrence. Binocularity status or stereoacuity changes were not associated with risk of recurrence. Significant numbers of children suffer recurrence of amblyopia after stopping therapy. Older age, better BCVA after stopping therapy and greater magnitude of improvement in BCVA are important risk factors for recurrence. Careful follow-up is essential for early detection and management of recurrence.

Factors predicting recurrence in successfully treated cases of anisometropic amblyopia

PubMed Central

Saxena, Rohit; Puranik, Shraddha; Singh, Digvijay; Menon, Vimla; Sharma, Pradeep; Phuljhele, Swati

2013-01-01

Context: Recurrence after successful treatment of amblyopia is known and understanding the risk factors could help effective management. Aim: To measure incidence of recurrence in successfully treated cases of anisometropic amblyopia and evaluate factors predicting it. Settings and Design: Cohort Study at a tertiary level institution. Materials and Methods: Successfully treated anisometropic amblyopes aged 4−12 years were followed up for 1 year after stopping therapy. Best corrected visual acuity (BCVA), refractive error, stereoacuity and contrast sensitivity were evaluated at baseline and follow-up. Statistical Analysis: Intergroup analysis with appropriate tests: Chi-square test, Fisher's exact test, Wilcoxon rank sum test and paired t-test. Results: One hundred and two patients with mean age at diagnosis 7.06 ± 1.81 years were followed-up for a mean duration of 1.0 ± 0.2 years. The mean pre-treatment BCVA (LogMAR score) at diagnosis was 0.73 ± 0.36 units which improved to 0.20 ± 0.00 with treatment and after 1 year of stopping treatment was 0.22 ± 0.07. Thirteen (12.74%) patients showed amblyopia recurrence during follow-up. Risk of recurrence was higher with older age of onset of treatment (6.64 ± 1.77 years without recurrence v/s 8.53 ± 1.39 years with recurrence, P = 0.0014). Greater extent of improvement of VA (P = 0.048) and final VA at stopping occlusion (P = 0.03) were associated with higher recurrence. Binocularity status or stereoacuity changes were not associated with risk of recurrence. Conclusions: Significant numbers of children suffer recurrence of amblyopia after stopping therapy. Older age, better BCVA after stopping therapy and greater magnitude of improvement in BCVA are important risk factors for recurrence. Careful follow-up is essential for early detection and management of recurrence. PMID:24343594

A Case of Ocular Toxocariasis Successfully Treated with Albendazole and Triamcinolon

PubMed Central

Seong, San; Moon, Daruchi; Lee, Dong Kyu; Kim, Hyung Eun; Oh, Hyun Sup; Kim, Soon Hyun; Kwon, Oh Woong

2014-01-01

We present a case of ocular toxocariasis treated successfully with oral albendazole in combination with steroids. A 26-year-old male visited the authors' clinic with the chief complaint of flying flies in his right eye. The fundus photograph showed a whitish epiretinal scar, and the fluorescein angiography revealed a hypofluorescein lesion of the scar and late leakage at the margin. An elevated retinal surface and posterior acoustic shadowing of the scar were observed in the optical coherence tomography, and Toxocara IgG was positive. The patient was diagnosed with toxocariasis, and the condition was treated with albendazole (400 mg twice a day) for a month and oral triamcinolone (16 mg for 2 weeks, once a day, and then 8 mg for 1 week, once a day) from day 13 of the albendazole treatment. The lesions decreased after the treatment. Based on this study, oral albendazole combined with steroids can be a simple and effective regimen for treating ocular toxocariasis. PMID:25352704

A case of ocular toxocariasis successfully treated with albendazole and triamcinolon.

PubMed

Seong, San; Moon, Daruchi; Lee, Dong Kyu; Kim, Hyung Eun; Oh, Hyun Sup; Kim, Soon Hyun; Kwon, Oh Woong; You, Yong Sung

2014-10-01

We present a case of ocular toxocariasis treated successfully with oral albendazole in combination with steroids. A 26-year-old male visited the authors' clinic with the chief complaint of flying flies in his right eye. The fundus photograph showed a whitish epiretinal scar, and the fluorescein angiography revealed a hypofluorescein lesion of the scar and late leakage at the margin. An elevated retinal surface and posterior acoustic shadowing of the scar were observed in the optical coherence tomography, and Toxocara IgG was positive. The patient was diagnosed with toxocariasis, and the condition was treated with albendazole (400 mg twice a day) for a month and oral triamcinolone (16 mg for 2 weeks, once a day, and then 8 mg for 1 week, once a day) from day 13 of the albendazole treatment. The lesions decreased after the treatment. Based on this study, oral albendazole combined with steroids can be a simple and effective regimen for treating ocular toxocariasis.

Establishment and Early Succession of Bacterial Communities in Monochloramine-treated Drinking Water Biofilms

EPA Science Inventory

The use of monochloramine as drinking water disinfectant is increasing because it forms lower levels of traditional disinfection by-products compared to free-chlorine. However, little is known about the bacterial succession within biofilms in monochloramine-treated systems. The d...

Ironing out the details of iron overload in myelofibrosis: Lessons from myelodysplastic syndromes.

PubMed

Carreau, Nicole; Tremblay, Douglas; Savona, Michael; Kremyanskaya, Marina; Mascarenhas, John

2016-09-01

Myelofibrosis (MF) and myelodysplastic syndrome (MDS) are hematopoietic stem cell disorders associated with cytopenias and red blood cell (RBC) transfusion dependence. Iron overload (IO) as a consequence of RBC transfusion dependence and its effect on outcomes in MF has not been formally studied. However, IO is a demonstrated poor prognostic feature in patients with MDS and congenital or acquired chronic anemias. Evidence that iron chelation therapy (ICT) reduces the deleterious effects of IO in MDS has led to speculation of benefit in MF. However, data supporting the use of ICT in MF is lacking. Neither disease has clear consensus guidelines for the use of ICT. Moreover, JAK-STAT inhibition, the cornerstone of MF treatment, often contributes to anemia and transfusional requirements. This manuscript reviews known and potential implications of IO in MF and highlights the need for prospective clinical investigations of ICT with consideration in the setting of JAK2 inhibitor therapy. Copyright © 2016 Elsevier Ltd. All rights reserved.

Clinical course and outcome of essential thrombocythemia and prefibrotic myelofibrosis according to the revised WHO 2016 diagnostic criteria

PubMed Central

Rumi, Elisa; Boveri, Emanuela; Bellini, Marta; Pietra, Daniela; Ferretti, Virginia V.; Sant’Antonio, Emanuela; Cavalloni, Chiara; Casetti, Ilaria C.; Roncoroni, Elisa; Ciboddo, Michele; Benvenuti, Pietro; Landini, Benedetta; Fugazza, Elena; Troletti, Daniela; Astori, Cesare; Cazzola, Mario

2017-01-01

The recently revised World Health Organization (WHO) classification of myeloid neoplasms recognizes prefibrotic myelofibrosis (prePMF) as a distinct entity, characterized by well-defined histopathologic features together with minor clinical criteria (leukocytes, anemia, increased LDH, splenomegaly). The aim of the study was to examine the clinical relevance of distinguishing prePMF from essential thrombocythemia (ET). We identified in our database all patients affected with ET, prePMF and primary myelofibrosis (PMF) diagnosed according to 2008 WHO criteria with a bone marrow fibrosis grade 0-1 at diagnosis and one DNA sample to define the mutational status. The bone marrow morphology of all 404 identified patients was reviewed by an expert pathologist and patients were reclassified according to the 2016 WHO criteria. After reclassification, our cohort included 269 ET, 109 prePMF, and 26 myeloproliferative neoplasm unclassificable. In comparison with ET, patients with prePMF had higher leukocyte count, lower hemoglobin level, higher platelet count, higher LDH values, and higher number of circulating CD34-positive cells; they showed more frequently splenomegaly (all P values < ·001). CALR mutations were more frequent in prePMF than in ET (35·8% vs 17·8%, P < ·001). PrePMF patients had shorter overall survival (P < ·001) and a trend to a higher incidence of leukemic evolution (P ·067) compared to ET patients, while they did not differ in terms of thrombotic and bleeding complications. In conclusion, ET and prePMF diagnosed according to 2016 WHO criteria are two entities with a different clinical phenotype at diagnosis and a different clinical outcome. PMID:29254200

Managing side effects of JAK inhibitors for myelofibrosis in clinical practice.

PubMed

Saeed, Iram; McLornan, Donal; Harrison, Claire N

2017-07-01

Myelofibrosis (MF) is characterized by bone marrow fibrosis, abnormalities in peripheral counts, extramedullary hematopoiesis, splenomegaly and an increased risk of transformation to acute myeloid leukaemia. The disease course is often heterogeneous and management can range from observation alone through to allogeneic stem cell transplantation. As of 2017, the only approved medication for MF remains the JAK Inhibitor (JAKi), ruxolitinib (Novartis Pharmaceuticals, Basel, Switzerland; Incyte, Wilmington, Detroit, USA) although several others have reached advanced stages of clinical trials. Areas covered: In this review, we focus on the management of both common and uncommon side effects arising from the use of currently approved and clinical trial JAKi. Most of the discussion concerns ruxolitinib although we also cover both pacritinib (CTI BioPharma) and momelotinib (Gilead Sciences, Foster City, California) which have been in recent large, multinational phase III trials. The various approaches to management of JAKi-related side effects are discussed - with particular emphasis to anaemia, thrombocytopaenia and infection risk. Expert commentary: JAK inhibitors are effective in many individuals with MF and have revolutionized the current treatment paradigm. The side effect profile, in the most, is predictable and manageable with high degrees of clinical surveillance and dose modifications.

Fecal Transplantation Successfully Treats Recurrent D-Lactic Acidosis in a Child With Short Bowel Syndrome.

PubMed

Davidovics, Zev H; Vance, Katherine; Etienne, Nancy; Hyams, Jeffrey S

2017-07-01

D-lactic acidosis can occur in patients with short bowel syndrome (SBS) when excessive malabsorbed carbohydrate (CHO) enters the colon and is metabolized by colonic bacteria to D-lactate. D-lactate can be absorbed systemically, and increased serum levels are associated with central nervous system toxicity manifested by confusion, ataxia, and slurred speech. Current therapy, usually directed toward suppressing intestinal bacterial overgrowth and limiting ingested CHO, is not always successful. Fecal transplantation, the infusion of donor feces into a recipient's intestinal tract, has been used for decades to treat recurrent Clostridium difficile infection, and case reports document its use in the successful treatment of constipation, diarrhea, and abdominal pain. The exact mechanism of action is unknown, but it is surmised that the alteration of the intestinal microbiome, as well as the reintroduction of potential beneficial microbes, helps mediate disease. Here we present the case of a child with SBS and recurrent, debilitating D-lactic acidosis, which was successfully treated with fecal transplantation.

Plasma cell cheilitis, successfully treated with topical 0.03% tacrolimus ointment.

PubMed

Jin, Seon Pil; Cho, Kwang Hyun; Huh, Chang Hun

2010-05-01

Plasma cell cheilitis is a rare, idiopathic mucosal condition. The treatment of plasma cell cheilitis is often disappointing. It is often resistant to various topical treatments. We present a 65-year-old woman who had a painful, eroded area on her lower lip, which responded poorly to various topical treatments. A biopsy revealed a band-like infiltration composed mainly of plasma cells in the dermis. She was diagnosed as having plasma cell cheilitis, and was successfully treated with 0.03% topical tacrolimus ointment.

Fludarabine Phosphate and Total Body Irradiation Followed by a Donor Peripheral Stem Cell Transplant in Treating Patients With Myelodysplastic Syndromes or Myeloproliferative Disorders

ClinicalTrials.gov

2018-03-28

Atypical Chronic Myeloid Leukemia, BCR-ABL1 Negative; Chronic Myelomonocytic Leukemia; de Novo Myelodysplastic Syndrome; Essential Thrombocythemia; Myeloproliferative Neoplasm; Paroxysmal Nocturnal Hemoglobinuria; Polycythemia Vera; Polycythemia Vera, Post-Polycythemic Myelofibrosis Phase; Primary Myelofibrosis; Refractory Anemia; Refractory Anemia With Excess Blasts; Refractory Anemia With Ring Sideroblasts; Refractory Cytopenia With Multilineage Dysplasia; Refractory Cytopenia With Multilineage Dysplasia and Ring Sideroblasts

Brief communication: Legionnaire's disease successfully treated in acute myelocytic leukemia during severe neutropenia.

PubMed

Guthrie, T H; Mahizhnan, P

1983-01-01

A patient with acute nonlymphocytic leukemia developed progressive lung infiltrates and unremitting fevers during a profound neutropenic state. Legionnaire's disease was diagnosed by simple immunologic studies and successfully treated with erythromycin. This index case alerts physicians toward a treatable infection which would not normally be susceptible to the empiric antibiotic regimens given neutropenic patients with fevers.

Legionella pneumophila pneumonia associated with acute respiratory distress syndrome successfully treated with imipenem.

PubMed

Mofredj, A; Baraka, D

1997-01-01

Although utility of beta-lactams in the treatment of legionnaire's disease could not be demonstrated, clinical cures of human legionellosis with imipenem have been reported. We report the case of a 45-year-old man who presented clinical and radiological features of 'typical' bacterial pneumonia. He was successfully treated with imipenem. Serologic studies showed seroconversion for Legionella pneumophila.

Pustular Palmoplantar Psoriasis Successfully Treated with Nb-UVB Monochromatic Excimer Light: A Case-Report.

PubMed

Gianfaldoni, Serena; Tchernev, Georgi; Wollina, Uwe; Lotti, Torello

2017-07-25

Barber's palmoplantar pustulosis (PPP) is a form of localised pustular psoriasis, affecting the palmar and plantar surfaces. It is a chronic disease, with a deep impact on the patients' quality of life. The Authors discuss a case of Baber Psoriasis successfully treated with monochromatic excimer light.

A Case of Refractory Pulmonary Coccidioidomycosis Successfully Treated with Posaconazole Therapy

PubMed Central

Patel, RH; Pandya, S; Nanjappa, S; Greene, JN

2018-01-01

Coccidioidomycosis is an endemic fungal infection caused by the inhalation of the spores of Coccidioides species. Patients with underlying immunosuppressive illness can contract chronic or disseminated disease which requires prolonged systemic therapy. Pulmonary coccidioidomycosis remains as an illusory and abstruse disease, with increased prevalence that poses as a challenge for clinicians in developing an effective strategy for treatment. Here, we report successful treatment of a refractory case of chronic relapsing pulmonary coccidioidomycosis in a 50-year old woman with a thin-walled cavitary lung lesion who was ultimately treated with posaconazole.

Pustular Palmoplantar Psoriasis Successfully Treated with Nb-UVB Monochromatic Excimer Light: A Case-Report

PubMed Central

Gianfaldoni, Serena; Tchernev, Georgi; Wollina, Uwe; Lotti, Torello

2017-01-01

Barber’s palmoplantar pustulosis (PPP) is a form of localised pustular psoriasis, affecting the palmar and plantar surfaces. It is a chronic disease, with a deep impact on the patients’ quality of life. The Authors discuss a case of Baber Psoriasis successfully treated with monochromatic excimer light. PMID:28785333

Successfully treated case of epithelioid sarcoma of the vulva.

PubMed

Chiyoda, Tatsuyuki; Ishikawa, Mitsuya; Nakamura, Masaru; Ogawa, Mariko; Takamatsu, Kiyoshi

2011-12-01

Epithelioid sarcoma of the vulva is an extremely rare and aggressive tumor. This tumor most commonly occurs on the labia majora in women of reproductive age. The molecular pathogenesis remains largely unknown. Only 20 cases of vulvar epithelioid sarcoma have been reported to date and more than half have had poor outcomes. We report a successfully treated case of vulvar epithelioid sarcoma in a 33-year-old woman. We performed a radical vulvectomy with flap reconstruction. Three years after surgery, the patient remains well, showing no evidence of recurrence. Early tissue diagnosis of vulvar epithelioid sarcoma is essential because this tumor can be clinically misdiagnosed as a benign lesion. Gynecologists should be aware of this rare tumor variant and carefully plan the treatment. © 2011 The Authors. Journal of Obstetrics and Gynaecology Research © 2011 Japan Society of Obstetrics and Gynecology.

A catheter-related bloodstream infection caused by Chryseobacterium indologenes successfully treated with antibiotic-lock rescue therapy.

PubMed

Corbella, Marta; Brandolini, Micaela; Cambieri, Patrizia; Decembrino, Nunzia; Pagani, Michele; Bottazzi, Andrea; Muzzi, Alba; Zecca, Marco; Mariani, Bianca; Marone, Piero

2017-07-01

We report the case of a catheter-related bloodstream infection caused by Chryseobacterium indologenes, an uncommon and multi-resistant pathogen, in a pediatric patient with a long-term vascular access device placed for chemotherapy treatment. The infection was successfully treated with ciprofloxacin antibiotic-lock therapy. This is the first report on successful salvage of a long-term device colonized by multi-resistant Chryseobacterium indologenes.

A case of candida albicans mediastinitis after heart transplantation successfully treated with caspofungin.

PubMed

Garlicki, Mirosław; Czub, Paweł; Filczak, Krzysztof; Wojdyga, Ryszard; Puchniewicz, Maciej; Labuś, Krzysztof; Ehrlich, Marek P

2006-01-01

Reported here is a case of mediastinitis caused by candida albicans and Staphylococcus aureus following a heart transplantation that was successfully treated with caspofungin, antibiotics and mediastinal lavage. A review of the literature revealed that Candida albicans as a cause of mediastinitis has been rarely described. In the few existing reports, evolution was generally fatal, especially in immunocompromised patients, despite treatment with antifungal drugs and antibiotics.

Loss of Ezh2 synergizes with JAK2-V617F in initiating myeloproliferative neoplasms and promoting myelofibrosis

PubMed Central

Nienhold, Ronny; Zmajkovic, Jakub; Hao-Shen, Hui; Geier, Florian; Dirnhofer, Stephan; Feenstra, Jelena D. Milosevic

2016-01-01

Myeloproliferative neoplasm (MPN) patients frequently show co-occurrence of JAK2-V617F and mutations in epigenetic regulator genes, including EZH2. In this study, we show that JAK2-V617F and loss of Ezh2 in hematopoietic cells contribute synergistically to the development of MPN. The MPN phenotype induced by JAK2-V617F was accentuated in JAK2-V617F;Ezh2−/− mice, resulting in very high platelet and neutrophil counts, more advanced myelofibrosis, and reduced survival. These mice also displayed expansion of the stem cell and progenitor cell compartments and a shift of differentiation toward megakaryopoiesis at the expense of erythropoiesis. Single cell limiting dilution transplantation with bone marrow from JAK2-V617F;Ezh2+/− mice showed increased reconstitution and MPN disease initiation potential compared with JAK2-V617F alone. RNA sequencing in Ezh2-deficient hematopoietic stem cells (HSCs) and megakaryocytic erythroid progenitors identified highly up-regulated genes, including Lin28b and Hmga2, and chromatin immunoprecipitation (ChIP)–quantitative PCR (qPCR) analysis of their promoters revealed decreased H3K27me3 deposition. Forced expression of Hmga2 resulted in increased chimerism and platelet counts in recipients of retrovirally transduced HSCs. JAK2-V617F–expressing mice treated with an Ezh2 inhibitor showed higher platelet counts than vehicle controls. Our data support the proposed tumor suppressor function of EZH2 in patients with MPN and call for caution when considering using Ezh2 inhibitors in MPN. PMID:27401344

A pooled analysis of overall survival in COMFORT-I and COMFORT-II, 2 randomized phase III trials of ruxolitinib for the treatment of myelofibrosis.

PubMed

Vannucchi, Alessandro M; Kantarjian, Hagop M; Kiladjian, Jean-Jacques; Gotlib, Jason; Cervantes, Francisco; Mesa, Ruben A; Sarlis, Nicholas J; Peng, Wei; Sandor, Victor; Gopalakrishna, Prashanth; Hmissi, Abdel; Stalbovskaya, Viktoriya; Gupta, Vikas; Harrison, Claire; Verstovsek, Srdan

2015-09-01

Ruxolitinib, a potent Janus kinase 1/2 inhibitor, resulted in rapid and durable improvements in splenomegaly and disease-related symptoms in the 2 phase III COMFORT studies. In addition, ruxolitinib was associated with prolonged survival compared with placebo (COMFORT-I) and best available therapy (COMFORT-II). We present a pooled analysis of overall survival in the COMFORT studies using an intent-to-treat analysis and an analysis correcting for crossover in the control arms. Overall, 301 patients received ruxolitinib (COMFORT-I, n=155; COMFORT-II, n=146) and 227 patients received placebo (n=154) or best available therapy (n=73). After a median three years of follow up, intent-to-treat analysis showed that patients who received ruxolitinib had prolonged survival compared with patients who received placebo or best available therapy [hazard ratio=0.65; 95% confidence interval (95%CI): 0.46-0.90; P=0.01]; the crossover-corrected hazard ratio was 0.29 (95%CI: 0.13-0.63). Both patients with intermediate-2- or high-risk disease showed prolonged survival, and patients with high-risk disease in the ruxolitinib group had survival similar to that of patients with intermediate-2-risk disease in the control group. The Kaplan-Meier estimate of overall survival at week 144 was 78% in the ruxolitinib arm, 61% in the intent-to-treat control arm, and 31% in the crossover-adjusted control arm. While larger spleen size at baseline was prognostic for shortened survival, reductions in spleen size with ruxolitinib treatment correlated with longer survival. These findings are consistent with previous reports and support that ruxolitinib offers a survival benefit for patients with myelofibrosis compared with conventional therapies. (clinicaltrials.gov identifiers: COMFORT-I, NCT00952289; COMFORT-II, NCT00934544). Copyright© Ferrata Storti Foundation.

Minocycline successfully treats exaggerated granulomatous hypersensitivity reaction to Mw immunotherapy.

PubMed

Vinay, Keshavamurthy; Narang, Tarun; Saikia, Uma N; Kumaran, Muthu Sendhil; Dogra, Sunil

2017-03-01

Mycobacterium W (Mw) vaccine has been found to be effective in the treatment of leprosy and warts. Despite increasing use of Mw immunotherapy, data on its safety is limited. We report a series of eight patients who developed persisting injection site granulomatous reaction following Mw immunotherapy and were successfully treated with minocycline. Eight patients with persistent nodular swelling at the site of Mw injections were identified. Seven of them had received Mw immunotherapy for cutaneous warts and one for verrucous epidermal nevus. The lesions were firm, erythematous, succulent, non-tender nodules confined to the sites of Mw vaccine injections. In 6 of these patients nodules also involved the previously injected areas. Skin biopsy from all patients showed eosinophil rich inflammation admixed with histiocytes and lymphocytes. In addition granulomas were seen in all with septal and nodular panniculitis in four patients. Broken and granular acid-fast bacilli were identified in two cases. All patients were treated with oral minocycline 100 mg/day for a mean of 9 weeks and showed good clinical response. Granulomatous reaction is a rare but significant adverse effect of Mw immunotherapy at cosmetically and functionally imperative sites. Oral minocycline appears to be effective therapy in this situation. © 2016 Wiley Periodicals, Inc.

A case of Diphyllobothrium nihonkaiense infection successfully treated by oral administration of Gastrografin.

PubMed

Yoshida, M; Hasegawa, H; Takaoka, H; Miyata, A

1999-08-01

A diphyllobothriid cestode infection found in a 54-year-old male residing in Oita, Japan, was successfully treated by oral administration of Gastrografin in combination with a intramuscular injection of Vagostigmin. The strobila expelled was 6.14 m long with a scolex, and morphologically identical with Diphyllobothrium nihonkaiense except unusual ovaries of which posterior horns were confluent in each proglottid. This is the first case of treatment of cestode infection by oral administration of Gastrografin.

Refractory case of adrenergic urticaria successfully treated with clotiazepam.

PubMed

Kawakami, Yukari; Gokita, Mari; Fukunaga, Atsushi; Nishigori, Chikako

2015-06-01

Adrenergic urticaria (AU) is a rare type of stress-induced physical urticaria characterized by widespread pruritic urticarial papules. Diagnosis can be made by i.d. injection of adrenaline or noradrenaline, which produces the characteristic rash. Although the lesions of AU typically respond to beta-blockers such as propranolol, the therapeutic options for AU are limited. Here, we report a case of AU that was resistant to beta-blockers and successfully treated with clotiazepam. The clinical picture of AU resembles that of cholinergic urticaria (CU), however, positive noradrenaline test and negative acetylcholine skin test were useful for the differential diagnosis of AU and CU. Although his symptoms were resistant to several therapeutic methods including olopatadine (H1 antagonist), lafutidine (H2 antagonist) and propranolol, the severity and frequency of his attacks and his subjective symptoms were reduced by oral clotiazepam, an anxiolytic benzodiazepine. Dermatologists should be aware that anxiolytic benzodiazepines may be a therapeutic option in AU. © 2015 Japanese Dermatological Association.

Superior vena cava syndrome with central venous catheter for chemotherapy treated successfully with fibrinolysis.

PubMed

Guijarro Escribano, J F; Antón, R F; Colmenarejo Rubio, A; Sáenz Cascos, L; Sainz González, F; Alguacil Rodríguez, R

2007-03-01

Recently, there has been an increase in the number of cases of superior vena cava (SVC) syndrome associated with chronic indwelling central venous catheters. Fibrinolytic therapy and endovascular treatment are currently achieving good results. We present a case history of a patient with SVC with a catheter used for chemotherapy, which was successfully treated with catheter-directed (intraclot) infusion thrombolytic therapy with urokinase.

Bedsores successfully treated with topical phenytoin.

PubMed

Inchingolo, Francesco; Vermesan, Dino; Inchingolo, Alessio D; Malcangi, Giuseppina; Santacroce, Luigi; Scacco, Salvatore; Benagiano, Vincenzo; Girolamo, Francesco; Cagiano, Raffaele; Caprio, Monica; Longo, Lucia; Abbinante, Antonia; Inchingolo, Angelo M; Dipalma, Gianna; Tarullo, Angelo; Tattoli, Maria

2017-04-28

Phenytoin is normally used in epilepsy treatment. One of the side effect affecting a significative part of the treated patients is the gingival overgrowth. It could surely be a correlation between this stimulatory effect and the assessment of phenytoin in wound healing. In fact, some studies of the literature have shown that topical phenytoin promotes healing of traumatic wounds, burns and ulcers by decubitus or stasis (diabetic or venous) and we emphasize, in vitiligo, a particular attention into repigmentation. The related mechanism of action seems to be multifactorial. In the present paper topical phenytoin has been used as wound-healing agent in 19 documented cases of bedsores, divided in treated and placebo group. The used concentration of phenytoin was 5 mg/L dissolved in a water solution of 9 g NaCl /L (0.9% P/V of NaCl). Patches soaked with phenytoin solution were applied over the bedsores along 3 hours every 12 hours. Results showed that phenytoin treated patients healed their wounds significantly before (p<0.001) with respect to controls.

Yield of prolonged wireless pH monitoring in achalasia patients successfully treated with pneumatic dilation

PubMed Central

Franchina, Marianna; Elvevi, Alessandra; Pugliese, Delia; Tenca, Andrea; Conte, Dario; Penagini, Roberto

2016-01-01

Background Gastro-oesophageal reflux disease (GORD) is a long-term complication of achalasia treatments. The aim of our study was to evaluate the yield of prolonged wireless pH monitoring in patients with successfully treated achalasia and its influence on proton pump inhibitor (PPI) use. Methods Twenty-five patients with achalasia who underwent prolonged wireless pH monitoring after a successful treatment with pneumatic dilation were enrolled. pH variables were analysed in the first 24 hours of monitoring to determine if tracings were indicative of GORD; the same variables were analysed in the following 24-hour period in order to obtain a worst-day diagnosis of GORD. PPI therapy before and after the test was recorded. Results Five out of 25 patients had GORD diagnosis during the first day of monitoring and four of them had oesophagitis at endoscopy. During the following days of monitoring four more patients had a diagnosis of GORD. Out of the 25 patients, PPIs were started after the test in six asymptomatic GORD-positive ones, whereas prescription of PPIs was stopped without detrimental effect on symptoms in three GORD-negative patients. Conclusions Prolonged wireless pH monitoring is a useful test to be added to endoscopy in order to evaluate GORD and to optimise antisecretory treatment in successfully treated achalasia patients. PMID:29026592

Use of liquid nitrogen and albendazole in successfully treating cutaneous larva migrans.

PubMed

Kapadia, Naseema; Borhany, Tasneem; Farooqui, Maria

2013-05-01

To determine the efficacy of combination treatment of Albendazole along with liquid nitrogen in cutaneous larva migrans. Quasi-experimental study. Abbasi Shaheed Hospital and The Aga Khan Hospital, Karachi, from December 2008 to December 2010. Eighteen cases of cutaneous larva migrans were collected and divided into two groups. Group-A was administered oral Albendazole 400 mg once per day along with topical steroid and oral cetrizine 10 mg once at night for 7 days. Group-B also received oral Albendazole 400 mg once per day along with cetrizine 10 mg once at night but they also received single application of liquid nitrogen to freeze the larva. It was found that in Group-A only 2 out of 9 (22%) showed improvement whereas 78% had to be given liquid nitrogen cryotherapy 3 - 7 days after Albendazole to prevent migration of larva. In Group-B, the improvement was 100% and all 9 patients were successfully treated. Use of liquid nitrogen along with oral anti-helminths is very effective in treating cutaneous larva migrans than Albendazole alone.

Immunology proves a great success for treating systemic autoimmune diseases - a perspective on immunopharmacology: IUPHAR Review 23.

PubMed

Ishii, Masaru

2017-07-01

Recent advances in the bioengineering of monoclonal antibodies (mAbs) have revolutionized the treatment of several immunological and rheumatic diseases. mAbs exhibit high specificity and affinity, and are very effective targeting agents, associated with minimal off-target adverse effects. Of several relevant immunological diseases, rheumatoid arthritis was the condition initially treated with mAbs, with great success. Currently, many immunological disorders are targeted and successfully treated using such novel approaches; these include inflammatory bowel diseases, multiple sclerosis, lupus and psoriasis. Today, the efforts of researchers in basic immunology (with a long history) have borne fruit; bioengineered mAbs are employed in clinical practice. In this brief review, I will describe the current and emerging therapeutic mAbs and molecular targeted agents, and discuss the future of the field, especially from the viewpoint of pharmacology. © 2017 The British Pharmacological Society.

Which patients with myelofibrosis should receive ruxolitinib therapy? ELN-SIE evidence-based recommendations.

PubMed

Marchetti, M; Barosi, G; Cervantes, F; Birgegård, G; Griesshammer, M; Harrison, C; Hehlmann, R; Kiladjian, J-J; Kröger, N; McMullin, M F; Passamonti, F; Vannucchi, A; Barbui, T

2017-04-01

Ruxolitinib is an oral Janus-activated kinase 1 (JAK1)/JAK2 inhibitor approved for the treatment of patients with myelofibrosis based on the results of two randomized clinical trials. However, discordant indications were provided by regulatory agencies and scientific societies for selecting the most appropriate candidates to this drug. The European LeukemiaNet and the Italian Society of Hematology shared the aim of building evidence-based recommendations for the use of ruxolitinib according to the GRADE methodology. Eighteen patient-intervention-comparator-outcome profiles were listed, each of them comparing ruxolitinib to other therapies with the aim of improving one of the three clinical outcomes: (a) splenomegaly, (b) disease-related symptoms, and (c) survival. Ruxolitinib was strongly recommended for improving symptomatic or severe (>15 cm below the costal margin) splenomegaly in patients with an International Prognostic Scoring System (IPSS)/dynamic IPSS risk intermediate 2 or high. Ruxolitinib was also strongly recommended for improving systemic symptoms in patients with an MPN10 score >44, refractory severe itching, unintended weight loss not attributable to other causes or unexplained fever. Because of weak evidence, the panel does not recommend ruxolitinib therapy for improving survival. Also, the recommendations given above do not necessarily apply to patients who are candidates for allogeneic stem cell transplant.

Childhood absence epilepsy successfully treated with the paleolithic ketogenic diet.

PubMed

Clemens, Zsófia; Kelemen, Anna; Fogarasi, András; Tóth, Csaba

2013-12-01

Childhood absence epilepsy is an epilepsy syndrome responding relatively well to the ketogenic diet with one-third of patients becoming seizure-free. Less restrictive variants of the classical ketogenic diet, however, have been shown to confer similar benefits. Beneficial effects of high fat, low-carbohydrate diets are often explained in evolutionary terms. However, the paleolithic diet itself which advocates a return to the human evolutionary diet has not yet been studied in epilepsy. Here, we present a case of a 7-year-old child with absence epilepsy successfully treated with the paleolithic ketogenic diet alone. In addition to seizure freedom achieved within 6 weeks, developmental and behavioral improvements were noted. The child remained seizure-free when subsequently shifted toward a paleolithic diet. It is concluded that the paleolithic ketogenic diet was effective, safe and feasible in the treatment of this case of childhood absence epilepsy.

Chronic catatonic schizophrenia treated successfully with right unilateral ultrabrief pulse electroconvulsive therapy: case report.

PubMed

Cupina, Denise; Patil, Sachin; Loo, Colleen

2013-06-01

Catatonia is a syndrome with prominent motor and behavioral symptoms commonly seen in acutely ill psychiatric patients. Catatonic symptoms have been considered as positive predictors of response to electroconvulsive therapy (ECT); however, few studies so far have addressed the role of ECT treatment technique in schizophrenia. We present the case of a 41-year-old woman with chronic catatonic schizophrenia who was treated successfully with a course of ultrabrief right unilateral ECT.

Isolated Bilateral Gastrocnemius Myositis in Crohn Disease Successfully Treated with Adalimumab.

PubMed

Vadala di Prampero, Salvatore; Marino, Marco; Toso, Francesco; Avellini, Claudio; Nguyen, Vu; Sorrentino, Dario

2016-01-01

Extraintestinal manifestations are common in inflammatory bowel disease; however, muscular involvement in Crohn disease is rarely reported. We present a case of a 26-year-old male with ileocolonic Crohn disease who developed sudden tenderness in both calves. Doppler ultrasound was negative for deep vein thrombosis. Magnetic resonance imaging of the gastrocnemius muscle showed high intensity signal in the muscle fibers, and muscle biopsy demonstrated nonspecific lymphocytic myositis. Other relevant laboratory results included normal antineutrophil cytoplasmic antibodies and creatine kinase as well as elevated C-reactive protein, erythrocyte sedimentation rate, and anti- Saccharomyces cerevisiae IgG titer. The patient was in clinical remission, being treated with azathioprine 2.5 mg/kg. Prednisone 60 mg/day was initiated with rapid resolution of calf tenderness; however, tenderness soon returned when the dose was tapered to 10 mg/day. Subsequently, prednisone and azathioprine were discontinued, and adalimumab was started at standard induction and maintenance doses. The patient's symptoms resolved shortly after the first induction dose. A repeat magnetic resonance imaging of the calves - 3 months after starting adalimumab - showed complete resolution of muscle inflammation. To our knowledge, this is the first case of gastrocnemius myositis - a rare extraintestinal manifestation of Crohn disease - successfully treated with anti-tumor necrosis factor agents.

Ruxolitinib: long-term management of patients with myelofibrosis and future directions in the treatment of myeloproliferative neoplasms.

PubMed

Yacoub, A; Odenike, O; Verstovsek, S

2014-12-01

Considerable clinical experience regarding the long-term efficacy and safety of ruxolitinib has been gathered since the drug was approved in the USA for patients with intermediate or high-risk myelofibrosis (MF) in November 2011. Findings from the pivotal phase 3 COMFORT studies showed that ruxolitinib-associated reductions in MF-related splenomegaly and symptom burden occur rapidly and in the majority of patients. Two- and 3-year follow-up data further suggest that the benefits of ruxolitinib are durable and associated with a survival advantage compared with conventional therapies. However, careful management of treatment-related thrombocytopenia and anemia with dose modifications and supportive care is critical to allow chronic therapy. Based on preliminary evidence, ruxolitinib also allows spleen size and symptom reduction before allogeneic stem cell transplantation without negative effect on engraftment or outcomes. In recent studies, ruxolitinib provided effective management of hematologic parameters and symptoms in patients with polycythemia vera refractory to or intolerant of hydroxyurea.

High absolute basophil count is a powerful independent predictor of inferior overall survival in patients with primary myelofibrosis.

PubMed

Lucijanic, Marko; Livun, Ana; Stoos-Veic, Tajana; Pejsa, Vlatko; Jaksic, Ozren; Cicic, David; Lucijanic, Jelena; Romic, Zeljko; Orehovec, Biserka; Aralica, Gorana; Miletic, Marko; Kusec, Rajko

2018-05-01

To investigate the clinical and prognostic significance of absolute basophil count (ABC) in patients with primary myelofibrosis (PMF). We retrospectively investigated 58 patients with PMF treated in our institution in the period from 2006 to 2017. ABC was obtained in addition to other hematological and clinical parameters. Patients were separated into high and low ABC groups using the Receiver operating characteristic curve analysis. ABC was higher in PMF patients than in healthy controls (P < 0.001). Patients with high ABC had higher white blood cells (P < 0.001), higher red cell distribution width (P = 0.035), higher lactate dehydrogenase (P < 0.001), more frequently had circulatory blasts (P < 0.001), constitutional symptoms (P = 0.030) and massive splenomegaly (P = 0.014). ABC was also positively correlated with absolute monocyte count (AMC) (P < 0.001) and other components of differential blood count. There was no difference in ABC regarding driver mutations or degree of bone marrow fibrosis. Univariately, high ABC was significantly associated with inferior overall survival (hazard ratio (HR) 4.79, P < 0.001). This effect remained statistically significant (HR 4.27, P = 0.009) in a multivariate Cox regression model adjusted for age, gender, Dynamic International Prognostic Scoring System (HR 2.6, P = 0.001) and AMC (HR 8.45, P = 0.002). High ABC reflects higher disease activity and stronger proliferative potential of disease. ABC and AMC independently predict survival and therefore seem to reflect different underlying pathophysiologic processes. Hence, both have a potential for improvement of current prognostic scores. Basophils represent a part of malignant clone in PMF and are associated with unfavorable disease features and poor prognosis which is independent of currently established prognostic scoring system and monocytosis.

A misdiagnosed Riedel's thyroiditis successfully treated by thyroidectomy and tamoxifen.

PubMed

Wang, Chih-Jung; Wu, Ta-Jen; Lee, Chung-Ta; Huang, Shih-Ming

2012-12-01

Riedel's thyroiditis, known as invasive fibrous thyroiditis, is a very rare form of chronic thyroiditis. It is hard to make the diagnosis without surgical biopsy. We present a case of Riedel's thyroiditis in a 52-year-old female with past history of Hashimoto's thyroiditis. She suffered from bilateral neck pain, which radiated to both lower jaws. The erythrocyte sedimentation rate was 125 mm/hour. Subacute thyroiditis superimposed on Hashimoto's thyroiditis was diagnosed and treated with steroid. However the response was poor and she had a history of severe peptic ulcer. To avoid inducing the peptic ulcer by steroid, she received bilateral subtotal thyroidectomy. During surgery, the thyroid had severe adhesion to surrounding soft tissue and the pathology showed Riedel's thyroiditis. The neck pain improved after thyroidectomy. Tamoxifen has been given for 8 months and the size of remnant thyroid decreased to 8 mm. We concluded that combined thyroidectomy and tamoxifen successfully cured a patient with Riedel's thyroiditis. Copyright © 2012. Published by Elsevier B.V.

Belinostat and Azacitidine in Treating Patients With Advanced Hematologic Cancers or Other Diseases

ClinicalTrials.gov

2014-12-22

Accelerated Phase of Disease; Adult Acute Myeloid Leukemia With Inv(16)(p13.1q22); CBFB-MYH11; Adult Acute Myeloid Leukemia With t(16;16)(p13.1;q22); CBFB-MYH11; Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); RUNX1-RUNX1T1; Adult Acute Myeloid Leukemia With t(9;11)(p22;q23); MLLT3-MLL; Adult Acute Promyelocytic Leukemia With t(15;17)(q22;q12); PML-RARA; Atypical Chronic Myeloid Leukemia, BCR-ABL1 Negative; Blastic Phase; Chronic Myelogenous Leukemia, BCR-ABL1 Positive; Chronic Myelomonocytic Leukemia; de Novo Myelodysplastic Syndrome; Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable; Philadelphia Chromosome Negative, BCR-ABL1 Positive Chronic Myelogenous Leukemia; Previously Treated Myelodysplastic Syndrome; Primary Myelofibrosis; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Disease; Secondary Acute Myeloid Leukemia; Secondary Myelodysplastic Syndrome

Ruxolitinib for the management of myelofibrosis: Results of an international physician survey.

PubMed

Ellis, Martin H; Koren-Michowitz, Maya; Lavi, Noa; Vannucchi, Alessandro M; Mesa, Ruben; Harrison, Claire N

2017-10-01

Ruxolitinib is established as treatment for symptomatic myeloproliferative neoplasm (MPN)-associated myelofibrosis. The strict inclusion and exclusion criteria and dose modification rules that applied to the COMFORTI and II studies that led to the licensing of ruxolitinib are not always applicable to routine clinical practice. Thus physicians now face decisions regarding ruxolitinib use that were not addressed in these pivotal trials. We performed an online survey of hematologists practicing in Europe, Israel, the United Kingdom and the United States. Demographic details regarding the physicians and their practice as relates to MPNs were collected. Management decisions pertaining to the use of ruxolitinib were obtained regarding 10 clinical scenarios relating to anemia, thrombocytopenia, frailty, infection and lack or loss of response to ruxolitnib in MF patients. 140 physicians responded to the survey. There were marked differences regarding their decisions for ruxolitinib administration in MF patients with or developing anemia or thrombocytopenia. Similarly there was little consensus regarding management of patients refractory or losing a response to ruxolitinib. There were differences between "MPN-focused" and "non-MPN-focused" physicians in certain areas. Physician practices regarding management of MF patients experiencing ruxolitinib-related toxicities or in whom response to the drug is lost was variable. This was true of "MPN-focused" and "non-MPN-focused" physicians in certain cases. Physician education and experience in using ruxolitinib may improve patient management. Copyright © 2017 Elsevier Ltd. All rights reserved.

Four cases of dysthymic disorder and general malaise successfully treated with traditional herbal (kampo) medicines: kamiuntanto.

PubMed

Kogure, Toshiaki; Tatsumi, Takeshi; Oku, Yuko

2010-01-01

Traditional herbal (Kampo) medicines have been used since ancient times to treat patients with mental disorders. In the present report, we describe four patients with dysthymia successfully treated with Kampo medicines: Kamiuntanto (KUT). These four patients fulfilled the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) criteria for dysthymic disorder with easy fatigability and sleeplessness, but did not fulfill the criteria for major depressive disorder. Treatment with KUT relieved depressive status, fatigue and sleeplessness in these patients. As a result, their QOL (quality of life) was considerably improved. KUT may be useful as an additional or alternative treatment for dysthymia, especially in the field of primary health care.

Four Cases of Dysthymic Disorder and General Malaise Successfully Treated with Traditional Herbal (Kampo) Medicines: Kamiuntanto

PubMed Central

Kogure, Toshiaki; Tatsumi, Takeshi; Oku, Yuko

2010-01-01

Traditional herbal (Kampo) medicines have been used since ancient times to treat patients with mental disorders. In the present report, we describe four patients with dysthymia successfully treated with Kampo medicines: Kamiuntanto (KUT). These four patients fulfilled the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV) criteria for dysthymic disorder with easy fatigability and sleeplessness, but did not fulfill the criteria for major depressive disorder. Treatment with KUT relieved depressive status, fatigue and sleeplessness in these patients. As a result, their QOL (quality of life) was considerably improved. KUT may be useful as an additional or alternative treatment for dysthymia, especially in the field of primary health care. PMID:21614161

An archetype of the collaborative efforts of psychotherapy and psychopharmacology in successfully treating dissociative identity disorder with comorbid bipolar disorder.

PubMed

Lakshmanan, Manu N; Meier, Stacey L Colton; Meier, Robert S; Lakshmanan, Ramaswamy

2010-07-01

We present a case where dissociative identity disorder was effectively treated with memory retrieval psychotherapy. However, the patient's comorbid bipolar disorder contributed to the patient's instability and fortified the amnesiac barriers that exist between alter personality states in dissociative identity disorder, which made memory retrieval difficult to achieve. Implications from this case indicate that a close collaboration between psychologist and psychiatrist focused on carefully diagnosing and treating existing comorbid conditions may be the most important aspect in treating dissociative identity disorder. We present our experience of successfully treating a patient with dissociative identity disorder and bipolar disorder using this collaborative method.

Two Cases of Refractory Cardiogenic Shock Secondary to Bupropion Successfully Treated with Veno-Arterial Extracorporeal Membrane Oxygenation.

PubMed

Heise, C William; Skolnik, Aaron B; Raschke, Robert A; Owen-Reece, Huw; Graeme, Kimberlie A

2016-09-01

Bupropion inhibits the uptake of dopamine and norepinephrine. Clinical effects in overdose include seizure, status epilepticus, tachycardia, arrhythmias, and cardiogenic shock. We report two cases of severe bupropion toxicity resulting in refractory cardiogenic shock, cardiac arrest, and repeated seizures treated successfully. Patients with cardiovascular failure related to poisoning may particularly benefit from extracorporeal membrane oxygenation (ECMO). These are the first cases of bupropion toxicity treated with veno-arterial EMCO (VA-ECMO) in which bupropion toxicity is supported by confirmatory testing. Both cases demonstrate the effectiveness of VA-ECMO in poisoned patients with severe cardiogenic shock or cardiopulmonary failure.

Temporal Trends in Helicobacter pylori Eradication Success in a Test-and-Treat Population.

PubMed

Boltin, Doron; Schmilovitz-Weiss, Hemda; Gingold-Belfer, Rachel; Leibovitzh, Haim; Snir, Yifat; Perets, Tsachi Tsadok; Dickman, Ram; Levi, Zohar; Niv, Yaron

2018-06-05

Although the efficacy of first-line treatment for Helicobacter pylori infection should aim to be > 90%, it is unclear whether this target has been achieved in Israel. We aimed to determine the success rate of treatment for H. pylori and to describe temporal changes in our region. Methods: Adult patients who underwent a first-time -C13-urea breath test (C13-UBT) at Clalit Health Services between January 1, 2010 and December 31, 2015 were included. In order to isolate a naïve "test-and-treat" population who were unlikely to have undergone an initial endoscopy-based H. pylori test, we excluded patients ≥45 years and those with any previous C13-UBT. A total of 94,590 subjects (36.1% male, age 28.5 ± 6.0) who underwent at least one C13-UBT during the study period were included. C13-UBT was positive in 48,509 (51.3%) subjects. A confirmatory post-treatment C13-UBT was performed in 37.8, 44.1, 46.6, and 45.9% following 1st, 2nd, 3rd, and 4th-line treatment respectively. Eradication was successful in 65.4% following first-line treatment, and eradication success improved during the study period (59.2, 63.3, 65.7, 66.0, 69.0, and 73.1% in 2010, 2011, 2012, 2013, 2014, and 2015 respectively; OR 1.11; 95% CI 1.09-1.13; p < 0.0001). Eradication was successful in 44.7% following second-line treatment, although eradication success did not significantly improve during the study period (OR 1.05; 95% CI 0.99-1.10; p = 0.09). Despite the increasing success of first-line treatment for H. pylori infection over the study period, eradication rates remain suboptimal. Initiatives to implement the Toronto and Maastricht Consensus Reports should be advanced. © 2018 S. Karger AG, Basel.

Sequential treatment of CD34+ cells from patients with primary myelofibrosis with chromatin-modifying agents eliminate JAK2V617F-positive NOD/SCID marrow repopulating cells

PubMed Central

Wang, Xiaoli; Zhang, Wei; Tripodi, Joseph; Lu, Min; Xu, Mingjiang; Najfeld, Vesna; Li, Yan

2010-01-01

Because primary myelofibrosis (PMF) originates at the level of the pluripotent hematopoietic stem cell (HSC), we examined the effects of various therapeutic agents on the in vitro and in vivo behavior of PMF CD34+ cells. Treatment of PMF CD34+ cells with chromatin-modifying agents (CMAs) but not hydroxyurea, Janus kinase 2 (JAK2) inhibitors, or low doses of interferon-α led to the generation of greater numbers of CD34+ chemokine (C-X-C motif) receptor (CXCR)4+ cells, which were capable of migrating in response to chemokine (C-X-C motif) ligand (CXCL)12 and resulted in a reduction in the proportion of hematopoietic progenitor cells (HPCs) that were JAK2V617F+. Furthermore, sequential treatment of PMF CD34+ cells but not normal CD34+ cells with decitabine (5-aza-2′-deoxycytidine [5azaD]), followed by suberoylanilide hydroxamic acid (SAHA; 5azaD/SAHA), or trichostatin A (5azaD/TSA) resulted in a higher degree of apoptosis. Two to 6 months after the transplantation of CMAs treated JAK2V617F+ PMF CD34+ cells into nonobese diabetic/severe combined immunodeficient (SCID)/IL-2Rγnull mice, the percentage of JAK2V617F/JAK2total in human CD45+ marrow cells was dramatically reduced. These findings suggest that both PMF HPCs, short-term and long-term SCID repopulating cells (SRCs), are JAK2V617F+ and that JAK2V617F+ HPCs and SRCs can be eliminated by sequential treatment with CMAs. Sequential treatment with CMAs, therefore, represents a possible effective means of treating PMF at the level of the malignant SRC. PMID:20858855

Aspergillus fumigatus Empyema, Arthritis, and Calcaneal Osteomyelitis in a Lung Transplant Patient Successfully Treated with Posaconazole

PubMed Central

Lodge, Barbara Alexander; Ashley, Elizabeth Dodds; Steele, Mark P.; Perfect, John R.

2004-01-01

A 64-year-old male with Aspergillus fumigatus infection that had disseminated from the lung to the ankle and adjacent bone was treated successfully with posaconazole after therapy with itraconazole and amphotericin B lipid complex failed. Marked clinical improvement occurred within 6 weeks of initiation of posaconazole therapy; after 6 months, infection had resolved at all sites. The patient has had no recurrence of infection. PMID:15004125

An Archetype of the Collaborative Efforts of Psychotherapy and Psychopharmacology in Successfully Treating Dissociative Identity Disorder with Comorbid Bipolar Disorder

PubMed Central

Lakshmanan, Manu N.; Meier, Stacey L. Colton; Meier, Robert S.

2010-01-01

We present a case where dissociative identity disorder was effectively treated with memory retrieval psychotherapy. However, the patient’s comorbid bipolar disorder contributed to the patient’s instability and fortified the amnesiac barriers that exist between alter personality states in dissociative identity disorder, which made memory retrieval difficult to achieve. Implications from this case indicate that a close collaboration between psychologist and psychiatrist focused on carefully diagnosing and treating existing comorbid conditions may be the most important aspect in treating dissociative identity disorder. We present our experience of successfully treating a patient with dissociative identity disorder and bipolar disorder using this collaborative method. PMID:20805917

Successive equimarginal approach for optimal design of a pump and treat system

NASA Astrophysics Data System (ADS)

Guo, Xiaoniu; Zhang, Chuan-Mian; Borthwick, John C.

2007-08-01

An economic concept-based optimization method is developed for groundwater remediation design. Design of a pump and treat (P&T) system is viewed as a resource allocation problem constrained by specified cleanup criteria. An optimal allocation of resources requires that the equimarginal principle, a fundamental economic principle, must hold. The proposed method is named successive equimarginal approach (SEA), which continuously shifts a pumping rate from a less effective well to a more effective one until equal marginal productivity for all units is reached. Through the successive process, the solution evenly approaches the multiple inequality constraints that represent the specified cleanup criteria in space and in time. The goal is to design an equal protection system so that the distributed contaminant plumes can be equally contained without bypass and overprotection is minimized. SEA is a hybrid of the gradient-based method and the deterministic heuristics-based method, which allows flexibility in dealing with multiple inequality constraints without using a penalty function and in balancing computational efficiency with robustness. This method was applied to design a large-scale P&T system for containment of multiple plumes at the former Blaine Naval Ammunition Depot (NAD) site, near Hastings, Nebraska. To evaluate this method, the SEA results were also compared with those using genetic algorithms.

[Incidence, associated factors, and follow-up of hospital heart failure complicating acute anterior myocardial infarction successfully treated by primary angioplasty].

PubMed

Meimoun, P; M'barek, D; Dragomir, C; Luycx-Bore, A; Elmkies, F; Boulanger, J; Zemir, H; Martis, S; Neykova, A; Tzvetkov, B; Clerc, J

2013-11-01

Heart failure (HF) complicating acute myocardial infarction (AMI) is of poor prognosis and is often associated with patient's characteristics and success of reperfusion strategies. However, few data is available regarding the high-risk subgroup of patients with anterior AMI treated successfully by primary angioplasty. The aim of the study was to assess the incidence, associated factors, and the future of HF occurring during hospitalisation, in the setting of anterior AMI treated successfully by primary angioplasty. Eighty-five consecutive patients with anterior AMI treated successfully by primary angioplasty (final angiographic TIMI flow grade=3, without residual stenosis) were included. Clinical, biochemical, angiographic, and echocardiographic data were prospectively collected and compared between patients with (Killip 2 and 3) and without HF during hospitalisation. Fifteen patients had HF (18%) during hospitalisation and 70 did not. By comparison to patients without HF, patients with HF were more frequently diabetics, had troponin peak and CPK, leucocytes count, and fasting glucose higher, LVEF and wall motion score index in the left anterior descending territory (WMSi-lad) poorer, and a lower non-invasive coronary flow reserve (CFR) in the LAD 24hours after angioplasty (all, P<0.05). In multivariate analysis, fasting glucose, leucocytes count after angioplasty, CFR and WMSi-lad were independently associated with HF, even after adjusting with angiographic variables (all, P<0.05). At 6months, patients with HF had less recovery of LV function and higher frequency of adverse LV remodelling (58% versus 20%, P<0.01) by comparison to patients without HF. In conclusion, HF is not uncommon even after successful primary angioplasty for anterior AMI (nearly one patient out of 5), is associated with hyperglycaemia and inflammation, a poor microvascular reperfusion, and left ventricular systolic function, and is more frequently complicated by adverse LV remodelling and lack

Scabies in a 2-month-old Infant Successfully Treated with Lindane.

PubMed

Jin, Seon Pil; Choi, Ji Eun; Won, Chong-Hyun; Cho, Soyun

2009-05-01

Diagnosis of scabies in young children can be challenging since the morphology and distribution of skin lesions may differ from adults. Therefore, clinicians should keep scabies in mind in their differential diagnosis in a child who presents with severe pruritic, polymorphic skin lesions. Regarding the treatment of scabies, the reported clinical experience with gamma benzene hexachloride (lindane) in young children is quite limited because of its neurotoxicity. However, a recent review suggests that lindane is an excellent alternative drug with minimal risk. We report the case of a 2-month-old male infant with pruritic, erythematous macules, papules, nodules, vesicles, and pustules from the top of the head to the tip of the toes. Initially, he was thought to have impetigo and antibiotics were prescribed. After obtaining a careful history and with the use of skin scraping, he was diagnosed with scabies. He was successfully treated with lindane with no adverse reactions.

Interphase FISH screening for the LCR-mediated common rearrangement of isochromosome 17q in primary myelofibrosis.

PubMed

Bien-Willner, Gabriel A; Stankiewicz, Paweł; Lupski, James R; Northup, Jill K; Velagaleti, Gopalrao V N

2005-08-01

Non-allelic homologous recombination (NAHR) between low-copy repeats (LCRs) has been implicated recently in somatic rearrangements including isochromosome i(17q), which is associated with hematologic malignancies as well as solid tumors. In hematological malignancies, the most common i(17q) breakpoint results from LCR-mediated NAHR, which creates a dicentric chromosome, idic(17)(p11.2). We report an elderly patient who presented with primary myelofibrosis (MF) with myeloid metaplasia (MMM), associated with idic(17)(p11.2) as the sole chromosomal abnormality, making this the first idic(17)(p11.2) myeloproliferative case reported in which the breakpoints are mapped to the breakpoint cluster region in proximal 17p. The rearrangement breakpoint maps to the previously defined LCR cluster, further suggesting that the genomic architecture of proximal 17p may be responsible for the formation of the majority of i(17q) cases. We describe our development of a rapid screening test using interphase FISH to detect idic(17)(p11.2), discuss the potential prognostic value of this molecular diagnostic test, and examine the relevance of LCR-mediated NAHR to common rearrangements in neoplasms. Copyright (c) 2005 Wiley-Liss, Inc.

Refractory major depression successfully treated with electroconvulsive therapy in a patient with Addison disease.

PubMed

Heijnen, Willemijn T C J; Pluijms, Esther M; Birkenhager, Tom K

2013-06-01

This report describes a 55-year-old woman who had 1 previous episode of major depression that responded favorably to treatment with tricyclic antidepressants. After the development of Addison disease, she experienced a new episode of major depression that failed to respond to adequate treatment with imipramine and was subsequently successfully treated with electroconvulsive therapy (ECT) with steroid cover. The patient did not experience adrenal crisis or adverse effects. After 9 ECT sessions, she attained full remission. These findings support the suggestion that ECT treatment is safe in patients with Addison disease when using 100 mg intravenous hydrocortisone as prophylaxis.

Mutant calreticulin knockin mice develop thrombocytosis and myelofibrosis without a stem cell self-renewal advantage.

PubMed

Li, Juan; Prins, Daniel; Park, Hyun Jung; Grinfeld, Jacob; Gonzalez-Arias, Carlos; Loughran, Stephen; Dovey, Oliver M; Klampfl, Thorsten; Bennett, Cavan; Hamilton, Tina L; Pask, Dean C; Sneade, Rachel; Williams, Matthew; Aungier, Juliet; Ghevaert, Cedric; Vassiliou, George S; Kent, David G; Green, Anthony R

2018-02-08

Somatic mutations in the endoplasmic reticulum chaperone calreticulin (CALR) are detected in approximately 40% of patients with essential thrombocythemia (ET) and primary myelofibrosis (PMF). Multiple different mutations have been reported, but all result in a +1-bp frameshift and generate a novel protein C terminus. In this study, we generated a conditional mouse knockin model of the most common CALR mutation, a 52-bp deletion. The mutant novel human C-terminal sequence is integrated into the otherwise intact mouse CALR gene and results in mutant CALR expression under the control of the endogenous mouse locus. CALR del/+ mice develop a transplantable ET-like disease with marked thrombocytosis, which is associated with increased and morphologically abnormal megakaryocytes and increased numbers of phenotypically defined hematopoietic stem cells (HSCs). Homozygous CALR del/del mice developed extreme thrombocytosis accompanied by features of MF, including leukocytosis, reduced hematocrit, splenomegaly, and increased bone marrow reticulin. CALR del/+ HSCs were more proliferative in vitro, but neither CALR del/+ nor CALR del/del displayed a competitive transplantation advantage in primary or secondary recipient mice. These results demonstrate the consequences of heterozygous and homozygous CALR mutations and provide a powerful model for dissecting the pathogenesis of CALR-mutant ET and PMF. © 2018 by The American Society of Hematology.

Protein kinase Cɛ inhibition restores megakaryocytic differentiation of hematopoietic progenitors from primary myelofibrosis patients.

PubMed

Masselli, E; Carubbi, C; Gobbi, G; Mirandola, P; Galli, D; Martini, S; Bonomini, S; Crugnola, M; Craviotto, L; Aversa, F; Vitale, M

2015-11-01

Among the three classic Philadelphia chromosome-negative myeloproliferative neoplasms, primary myelofibrosis (PMF) is the most severe in terms of disease biology, survival and quality of life. Abnormalities in the process of differentiation of PMF megakaryocytes (MKs) are a hallmark of the disease. Nevertheless, the molecular events that lead to aberrant megakaryocytopoiesis have yet to be clarified. Protein kinase Cɛ (PKCɛ) is a novel serine/threonine kinase that is overexpressed in a variety of cancers, promoting aggressive phenotype, invasiveness and drug resistance. Our previous findings on the role of PKCɛ in normal (erythroid and megakaryocytic commitment) and malignant (acute myeloid leukemia) hematopoiesis prompted us to investigate whether it could be involved in the pathogenesis of PMF MK-impaired differentiation. We demonstrate that PMF megakaryocytic cultures express higher levels of PKCɛ than healthy donors, which correlate with higher disease burden but not with JAK2V617F mutation. Inhibition of PKCɛ function (by a negative regulator of PKCɛ translocation) or translation (by target small hairpin RNA) leads to reduction in PMF cell growth, restoration of PMF MK differentiation and inhibition of PKCɛ-related anti-apoptotic signaling (Bcl-xL). Our data suggest that targeting PKCɛ directly affects the PMF neoplastic clone and represent a proof-of-concept for PKCɛ inhibition as a novel therapeutic strategy in PMF.

Malignant lymphoma of the vagina successfully treated with rituximab, adryamicin, cyclophosphamide, vincristine sulfate, and prednisolone.

PubMed

Nasu, K; Okamoto, M; Nishida, M; Takai, N; Narahara, H

2012-01-01

Primary malignant lymphoma of the vagina is extremely rare. The most common histologic subtype is diffuse large B-cell lymphoma (DLBCL). We report a case of vaginal DLBCL successfully treated with chemotherapy consisting of rituximab, adryamicin, cyclophosphamide, vincristine sulfate, and prednisolone (R-CHOP), followed by pelvic irradiation. A 44-year-old Japanese woman was admitted complaining of atypical genital bleeding and puruloid vaginal discharge. Gynecological examination showed an ulceration of the vaginal wall and a hard mass the size of a goose egg beneath the left vaginal wall, which had infiltrated to the left pelvic wall. The pathological diagnosis based on a punch biopsy taken from the vaginal tumor was non-Hodgkin's lymphoma. Based on immunohistochemical study, the tumor was subclassified as activated B-cell type DLBCL. The patient was diagnosed with Ann Arbor Stage IEA DLBCL and Stage III vaginal cancer, according to the International Federation of Gynecologists and Obstetricians (FIGO) classification system. She was successfully treated by six courses of R-CHOP, followed by radiation therapy. The patient is well without evidence of disease 13 months following the initial treatment. Little attention has been paid to the use of rituximab in addition to conventional chemotherapy and the importance of clinical and morphological subgrouping of DLBCL arising in the vagina. The present case indicates that the effects of rituximab on the prognosis of vaginal DLBCL must be evaluated, and that clinical use of immunophenotypic subgrouping should be considered for vaginal DLBCL.

Lyme carditis with isolated left bundle branch block and myocarditis successfully treated with oral doxycycline.

PubMed

Cunha, Burke A; Elyasi, Maekal; Singh, Prince; Jimada, Ismail

2018-01-01

Lyme disease may present with a variety of cardiac manifestations ranging from first degree to third degree heart block. Cardiac involvement with Lyme disease may be asymptomatic, or symptomatic. Atrioventrical conduction abnormalities are the most common manifestation of Lyme carditis. Less common, are alternating right bundle branch block (RBBB) and left bundle branch block (LBBB). We present an interesting case of a young male whose main manifestation of Lyme carditis was isolated LBBB. He also had mild Lyme myocarditis. The patient was successfully treated with oral doxycycline, and his isolated LBBB and myocarditis rapidly resolved.

Stretching the indications: high tibial osteotomy used successfully to treat isolated ankle symptoms

PubMed Central

Elson, David W; Paweleck, James E; Shields, David W; Dawson, Matthew J; Ferrier, Gail M

2013-01-01

High tibial osteotomy (HTO) is successful in treating symptomatic varus arthritis of the knee. We present a case where ankle pain and instability were attributed to varus ankle malalignment. This was found to be secondary to constitutional varus of the proximal tibia but the patient's knee was asymptomatic. The decision to operate on an asymptomatic knee in the hope of improving ankle symptoms took a period of careful consideration, planning and discussion. HTO was performed without immediate complication and the patient reported an excellent outcome with marked improvement in Mazur's foot and ankle score from 18 to 85. In well selected and planned cases, HTO may be considered as an instrument of deformity correction with improvement in symptoms from joints distant to the surgical site. PMID:24022901

Lumbar arthrodesis infection by multi-resistant Klebsiella pneumoniae, successfully treated with implant retention and ceftazidime/avibactam.

PubMed

Rico-Nieto, A; Moreno-Ramos, F; Fernández-Baillo, N

2018-04-07

There are increasingly more patients with prosthetic implants (orthopaedic prostheses, lumbar instruments, osteosynthesis material). In the last decade, infections caused by carbapenem resistant Enterobacteriaceae have increased (bacteriaemia, abscesses, urinary tract infections...) with great difficulty in treatment and important associated comorbidity. We present the first case of infection of a lumbar instrumentation by Klebsiella pneumoniae producing carbapenemase D, OXA-48 type, and successfully treated. Copyright © 2018 SECOT. Publicado por Elsevier España, S.L.U. All rights reserved.

A 7-Gene Signature Depicts the Biochemical Profile of Early Prefibrotic Myelofibrosis

PubMed Central

Skov, Vibe; Burton, Mark; Thomassen, Mads; Stauffer Larsen, Thomas; Riley, Caroline H.; Brinch Madelung, Ann; Kjær, Lasse; Bondo, Henrik; Stamp, Inger; Ehinger, Mats; Dahl-Sørensen, Rasmus; Brochmann, Nana; Nielsen, Karsten; Thiele, Jürgen; Jensen, Morten K.; Weis Bjerrum, Ole; Kruse, Torben A.; Hasselbalch, Hans Carl

2016-01-01

Recent studies have shown that a large proportion of patients classified as essential thrombocythemia (ET) actually have early primary prefibrotic myelofibrosis (prePMF), which implies an inferior prognosis as compared to patients being diagnosed with so-called genuine or true ET. According to the World Health Organization (WHO) 2008 classification, bone marrow histology is a major component in the distinction between these disease entities. However, the differential diagnosis between them may be challenging and several studies have not been able to distinguish between them. Most lately, it has been argued that simple blood tests, including the leukocyte count and plasma lactate dehydrogenase (LDH) may be useful tools to separate genuine ET from prePMF, the latter disease entity more often being featured by anemia, leukocytosis and elevated LDH. Whole blood gene expression profiling was performed in 17 and 9 patients diagnosed with ET and PMF, respectively. Using elevated LDH obtained at the time of diagnosis as a marker of prePMF, a 7-gene signature was identified which correctly predicted the prePMF group with a sensitivity of 100% and a specificity of 89%. The 7 genes included MPO, CEACAM8, CRISP3, MS4A3, CEACAM6, HEMGN, and MMP8, which are genes known to be involved in inflammation, cell adhesion, differentiation and proliferation. Evaluation of bone marrow biopsies and the 7-gene signature showed a concordance rate of 71%, 79%, 62%, and 38%. Our 7-gene signature may be a useful tool to differentiate between genuine ET and prePMF but needs to be validated in a larger cohort of “ET” patients. PMID:27579896

Concurrent MPL515 and JAK2V617F mutations in myelofibrosis: chronology of clonal emergence and changes in mutant allele burden over time.

PubMed

Lasho, Terra L; Pardanani, Animesh; McClure, Rebecca F; Mesa, Ruben A; Levine, Ross L; Gilliland, D Gary; Tefferi, Ayalew

2006-12-01

MPLW515L/K and JAK2V617F can co-exist in myelofibrosis with myeloid metaplasia (MMM). The chronology of clonal emergence was studied in three such cases using serially stored bone marrow. At diagnosis, a major MPL515 mutant clone was accompanied by a minor JAK2V617F clone in all three instances. At 25 time points over a period of 4-8 years, allele burden fluctuated but remained high for MPLW515L/K and low for JAK2V617F. We conclude that MPLW515L/K and JAK2V617F are both early events in MMM and allele burden, rather than the mere presence of these mutations, might be relevant to phenotypic variation in myeloproliferative disorders.

Chronic thrombotic obstruction of major pulmonary arteries : report of a case successfully treated by thrombendarterectomy, and a review of the literature.

DOT National Transportation Integrated Search

1963-08-01

A case is reported of chronic massive thromboembolic occlusion of major pulmonary arterial branches successfully treated with embolectomy and endarterectomy. The historic features, physical signs and pathophysiologic alterations which form the basis ...

Plasma radiofrequency preceded by pressure recording enhances success for treating sleep-related breathing disorders.

PubMed

Tvinnereim, Magne; Mitic, Svetislav; Hansen, Rolf K

2007-04-01

Sleep-related breathing disorders (SRBD) that cannot be treated conservatively are commonly treated using uvulopalatopharyngoplasty, although success rates are generally less than 70%. The purpose of this study was to assess a plasma-mediated radiofrequency (RF)-based coblation assisted upper airway procedure (CAUP) to treat SRBD patients determined to have obstruction localized to the upper (mid)-pharyngeal region. Prospective case series (n = 40). Six patients had socially bothersome snoring, and 34 patients had mild to moderate sleep apnea (apnea/hypopnea index [AHI] <20), with the primary level of obstruction (>50%) within the upper pharyngeal region as determined using whole night recordings, including airway pressure fluctuation monitoring (ApneaGraph, MRA-Medical Ltd, Gloucestershire, UK). CAUP consisted of making a lateral palatal incision, ablating three upward channels on each side of the midline (fan-shaped) into the soft palate using a plasma mediated RF-based device (ArthroCare Corporation, Austin, TX), and performing a partial uvulectomy. Clinical outcomes included the Epworth Sleepiness Scale (ESS), partner rating of snoring using a visual analogue scale (VAS), and night-time apnea and hypopnea events (AHI, hypopnea index [HI], apnea index [AI]). Patients were 28 to 68 (46 +/- 12) years old; 28 (70%) were male. Preoperatively, clinical assessment scores (median +/- interquartile range) were as follows: ESS (11.0 +/- 3.0), VAS (8.15 +/- 1.00), AHI (9.58 +/- 5.58), HI (9.00 +/- 5.29), AI (0.333 +/- 0.625). After CAUP, no postoperative scarring, fibrosis, or any other clinically significant side effects were observed. Postoperatively (9.1 +/- 1.5; 7-15 mo), ESS (4.0 +/- 1.0), VAS (2.70 +/- 1.38), AHI (3.75 +/- 2.92), HI (3.58 +/- 2.50), and AI (0.167 +/- 0.167) were significantly improved (P < .001). CAUP preceded by site-specific obstruction diagnosis using pressure recording is a well-tolerated outpatient treatment that is well suited for treating

Clinical effect of driver mutations of JAK2, CALR, or MPL in primary myelofibrosis.

PubMed

Rumi, Elisa; Pietra, Daniela; Pascutto, Cristiana; Guglielmelli, Paola; Martínez-Trillos, Alejandra; Casetti, Ilaria; Colomer, Dolors; Pieri, Lisa; Pratcorona, Marta; Rotunno, Giada; Sant'Antonio, Emanuela; Bellini, Marta; Cavalloni, Chiara; Mannarelli, Carmela; Milanesi, Chiara; Boveri, Emanuela; Ferretti, Virginia; Astori, Cesare; Rosti, Vittorio; Cervantes, Francisco; Barosi, Giovanni; Vannucchi, Alessandro M; Cazzola, Mario

2014-08-14

We studied the impact of driver mutations of JAK2, CALR, (calreticulin gene) or MPL on clinical course, leukemic transformation, and survival of patients with primary myelofibrosis (PMF). Of the 617 subjects studied, 399 (64.7%) carried JAK2 (V617F), 140 (22.7%) had a CALR exon 9 indel, 25 (4.0%) carried an MPL (W515) mutation, and 53 (8.6%) had nonmutated JAK2, CALR, and MPL (so-called triple-negative PMF). Patients with CALR mutation had a lower risk of developing anemia, thrombocytopenia, and marked leukocytosis compared with other subtypes. They also had a lower risk of thrombosis compared with patients carrying JAK2 (V617F). At the opposite, triple-negative patients had higher incidence of leukemic transformation compared with either CALR-mutant or JAK2-mutant patients. Median overall survival was 17.7 years in CALR-mutant, 9.2 years in JAK2-mutant, 9.1 years in MPL-mutant, and 3.2 years in triple-negative patients. In multivariate analysis corrected for age, CALR-mutant patients had better overall survival than either JAK2-mutant or triple-negative patients. The impact of genetic lesions on survival was independent of current prognostic scoring systems. These observations indicate that driver mutations define distinct disease entities within PMF. Accounting for them is not only relevant to clinical decision-making, but should also be considered in designing clinical trials. © 2014 by The American Society of Hematology.

Clinical effect of driver mutations of JAK2, CALR, or MPL in primary myelofibrosis

PubMed Central

Rumi, Elisa; Pietra, Daniela; Pascutto, Cristiana; Guglielmelli, Paola; Martínez-Trillos, Alejandra; Casetti, Ilaria; Colomer, Dolors; Pieri, Lisa; Pratcorona, Marta; Rotunno, Giada; Sant’Antonio, Emanuela; Bellini, Marta; Cavalloni, Chiara; Mannarelli, Carmela; Milanesi, Chiara; Boveri, Emanuela; Ferretti, Virginia; Astori, Cesare; Rosti, Vittorio; Cervantes, Francisco; Barosi, Giovanni; Vannucchi, Alessandro M.

2014-01-01

We studied the impact of driver mutations of JAK2, CALR, (calreticulin gene) or MPL on clinical course, leukemic transformation, and survival of patients with primary myelofibrosis (PMF). Of the 617 subjects studied, 399 (64.7%) carried JAK2 (V617F), 140 (22.7%) had a CALR exon 9 indel, 25 (4.0%) carried an MPL (W515) mutation, and 53 (8.6%) had nonmutated JAK2, CALR, and MPL (so-called triple-negative PMF). Patients with CALR mutation had a lower risk of developing anemia, thrombocytopenia, and marked leukocytosis compared with other subtypes. They also had a lower risk of thrombosis compared with patients carrying JAK2 (V617F). At the opposite, triple-negative patients had higher incidence of leukemic transformation compared with either CALR-mutant or JAK2-mutant patients. Median overall survival was 17.7 years in CALR-mutant, 9.2 years in JAK2-mutant, 9.1 years in MPL-mutant, and 3.2 years in triple-negative patients. In multivariate analysis corrected for age, CALR-mutant patients had better overall survival than either JAK2-mutant or triple-negative patients. The impact of genetic lesions on survival was independent of current prognostic scoring systems. These observations indicate that driver mutations define distinct disease entities within PMF. Accounting for them is not only relevant to clinical decision-making, but should also be considered in designing clinical trials. PMID:24986690

Late recurrence of nonseminomatous germ cell tumor successfully treated with intensity-modulated radiation therapy.

PubMed

Kita, Yuki; Imamura, Masaaki; Mizowaki, Takashi; Norihisa, Yoshiki; Yoshimura, Koji; Hiraoka, Masahiro; Ogawa, Osamu

2013-08-01

We report the case of a 41-year-old man with a late recurrence of nonseminomatous germ cell tumor, which was successfully treated with intensity-modulated radiation therapy. For the residual retrocrural tumor invading the 11th and 12th thoracic vertebrae with an abnormal level of tumor marker (α-fetoprotein: 23.2 ng/ml) after salvage chemotherapy, chemotherapy could not be continued due to its neurotoxicity, and surgery could not be performed due to the location. In this situation, intensity-modulated radiation therapy achieved a complete response of tumor marker. The patient remained in complete clinical remission after 3 years. The efficacy of radiotherapy, especially intensity-modulated radiation therapy, for a nonseminomatous germ cell tumor is discussed.

Tie2 Expressing Monocytes in the Spleen of Patients with Primary Myelofibrosis

PubMed Central

Campanelli, Rita; Fois, Gabriela; Catarsi, Paolo; Poletto, Valentina; Villani, Laura; Erba, Benedetta Gaia; Maddaluno, Luigi; Jemos, Basilio; Salmoiraghi, Silvia; Guglielmelli, Paola; Abbonante, Vittorio; Di Buduo, Christian Andrea; Balduini, Alessandra; Iurlo, Alessandra; Barosi, Giovanni; Rosti, Vittorio; Massa, Margherita

2016-01-01

Primary myelofibrosis (PMF) is a Philadelphia-negative (Ph−) myeloproliferative disorder, showing abnormal CD34+ progenitor cell trafficking, splenomegaly, marrow fibrosis leading to extensive extramedullary haematopoiesis, and abnormal neoangiogenesis in either the bone marrow or the spleen. Monocytes expressing the angiopoietin-2 receptor (Tie2) have been shown to support abnormal angiogenic processes in solid tumors through a paracrine action that takes place in proximity to the vessels. In this study we investigated the frequency of Tie2 expressing monocytes in the spleen tissue samples of patients with PMF, and healthy subjects (CTRLs), and evaluated their possible role in favouring spleen angiogenesis. We show by confocal microscopy that in the spleen tissue of patients with PMF, but not of CTRLs, the most of the CD14+ cells are Tie2+ and are close to vessels; by flow cytometry, we found that Tie2 expressing monocytes were Tie2+CD14lowCD16brightCDL62−CCR2− (TEMs) and their frequency was higher (p = 0.008) in spleen tissue-derived mononuclear cells (MNCs) of patients with PMF than in spleen tissue-derived MNCs from CTRLs undergoing splenectomy for abdominal trauma. By in vitro angiogenesis assay we evidenced that conditioned medium of immunomagnetically selected spleen tissue derived CD14+ cells of patients with PMF induced a denser tube like net than that of CTRLs; in addition, CD14+Tie2+ cells sorted from spleen tissue derived single cell suspension of patients with PMF show a higher expression of genes involved in angiogenesis than that found in CTRLs. Our results document the enrichment of Tie2+ monocytes expressing angiogenic genes in the spleen of patients with PMF, suggesting a role for these cells in starting/maintaining the pathological angiogenesis in this organ. PMID:27281335

Hyponatremia due to Secondary Adrenal Insufficiency Successfully Treated by Dexamethasone with Sodium Chloride

PubMed Central

Kazama, Itsuro; Tamada, Tsutomu; Nakajima, Toshiyuki

2015-01-01

Patient: Female, 60 Final Diagnosis: Hyponatremia due to secondary adrenal insufficiency Symptoms: prolonged general fatigue and anorexia Medication: — Clinical Procedure: Successfully treated by dexamethasone with sodium chloride Specialty: Nephrology Objective: Rare co-existance of disease or pathology Background: Patients who were surgically treated for Cushing’s syndrome postoperatively surrender to “primary” adrenal insufficiency. However, the preoperative over-secretion of cortisol or the postoperative administration of excessive glucocorticoids can cause “secondary” adrenal insufficiency, in which the prevalence of hyponatremia is usually lower than that of primary adrenal insufficiency. Case Report: A 60-year-old woman with a past medical history of Cushing’s syndrome developed hyponatremia with symptoms of acute glucocorticoid deficiency, such as prolonged general fatigue and anorexia, after upper respiratory tract infection. A decrease in the serum cortisol level and the lack of increase in the ACTH level, despite the increased demand for cortisol, enabled a diagnosis of “secondary” adrenal insufficiency. Although the initial fluid replacement therapy was not effective, co-administration of dexamethasone and sodium chloride quickly resolved her symptoms and ameliorated the refractory hyponatremia. Conclusions: In this case, the hypothalamic-pituitary axis of the patient was thought to have become suppressed long after the surgical treatment for Cushing’s syndrome. This case suggested a mechanism of refractory hyponatremia caused by secondary adrenal insufficiency, for which the administration of dexamethasone and sodium chloride exerted additional therapeutic efficacy. PMID:26319655

Gastric and enteric anisakiasis successfully treated with Gastrografin therapy: A case report.

PubMed

Fujikawa, Hiroki; Kuwai, Toshio; Yamaguchi, Toshiki; Miura, Ryoichi; Sumida, Yuki; Takasago, Takeshi; Miyasako, Yuki; Nishimura, Tomoyuki; Iio, Sumio; Imagawa, Hiroki; Yamaguchi, Atsushi; Kouno, Hirotaka; Kohno, Hiroshi

2018-03-16

We report a case of a 59-year-old woman who was diagnosed with gastric and small intestinal anisakiasis, which was successfully treated with endoscopic extraction and Gastrografin therapy. She was admitted to our hospital with epigastric pain and vomiting one day after eating raw fish. She exhibited tenderness in the epigastrium without obvious rebound tenderness or guarding. Computed tomography (CT) demonstrated segmental edema of the intestinal wall with proximal dilatation and a small number of ascites. Because enteric anisakiasis was suspected based on the patient's history of recent raw fish consumption and abdominal CT, we performed gastroscopy and confirmed that nine Anisakis larvae were attached to the gastric mucosa. All of the Anisakis larvae were extracted via endoscopy, and the patient was diagnosed with gastric and enteric anisakiasis. Additionally, in the hospital, we performed ileography twice using Gastrografin, which led to shortened hospital stay. Based on the clinical results of this case, we suggest that Gastrografin therapy is a safe, convenient, and useful method to extract enteric Anisakis larvae.

Integrative analysis of copy number and gene expression data suggests novel pathogenetic mechanisms in primary myelofibrosis.

PubMed

Salati, Simona; Zini, Roberta; Nuzzo, Simona; Guglielmelli, Paola; Pennucci, Valentina; Prudente, Zelia; Ruberti, Samantha; Rontauroli, Sebastiano; Norfo, Ruggiero; Bianchi, Elisa; Bogani, Costanza; Rotunno, Giada; Fanelli, Tiziana; Mannarelli, Carmela; Rosti, Vittorio; Salmoiraghi, Silvia; Pietra, Daniela; Ferrari, Sergio; Barosi, Giovanni; Rambaldi, Alessandro; Cazzola, Mario; Bicciato, Silvio; Tagliafico, Enrico; Vannucchi, Alessandro M; Manfredini, Rossella

2016-04-01

Primary myelofibrosis (PMF) is a Myeloproliferative Neoplasm (MPN) characterized by megakaryocyte hyperplasia, progressive bone marrow fibrosis, extramedullary hematopoiesis and transformation to Acute Myeloid Leukemia (AML). A number of phenotypic driver (JAK2, CALR, MPL) and additional subclonal mutations have been described in PMF, pointing to a complex genomic landscape. To discover novel genomic lesions that can contribute to disease phenotype and/or development, gene expression and copy number signals were integrated and several genomic abnormalities leading to a concordant alteration in gene expression levels were identified. In particular, copy number gain in the polyamine oxidase (PAOX) gene locus was accompanied by a coordinated transcriptional up-regulation in PMF patients. PAOX inhibition resulted in rapid cell death of PMF progenitor cells, while sparing normal cells, suggesting that PAOX inhibition could represent a therapeutic strategy to selectively target PMF cells without affecting normal hematopoietic cells' survival. Moreover, copy number loss in the chromatin modifier HMGXB4 gene correlates with a concomitant transcriptional down-regulation in PMF patients. Interestingly, silencing of HMGXB4 induces megakaryocyte differentiation, while inhibiting erythroid development, in human hematopoietic stem/progenitor cells. These results highlight a previously un-reported, yet potentially interesting role of HMGXB4 in the hematopoietic system and suggest that genomic and transcriptional imbalances of HMGXB4 could contribute to the aberrant expansion of the megakaryocytic lineage that characterizes PMF patients. © 2015 UICC.

Topotecan Hydrochloride and Carboplatin With or Without Veliparib in Treating Advanced Myeloproliferative Disorders and Acute Myeloid Leukemia or Chronic Myelomonocytic Leukemia

ClinicalTrials.gov

2018-06-22

Acute Myeloid Leukemia; Acute Myeloid Leukemia Arising From Previous Myelodysplastic Syndrome; Atypical Chronic Myeloid Leukemia, BCR-ABL1 Negative; Chronic Myelomonocytic Leukemia; Essential Thrombocythemia; Myelodysplastic/Myeloproliferative Neoplasm; Myelofibrosis; Polycythemia Vera; Recurrent Adult Acute Myeloid Leukemia; Refractory Acute Myeloid Leukemia

Elevated compartment pressures from copperhead envenomation successfully treated with antivenin.

PubMed

Mazer-Amirshahi, Maryann; Boutsikaris, Amy; Clancy, Cathleen

2014-01-01

Copperhead envenomation causes local soft tissue effects; however, associated compartment syndrome is rare. We report a case of a 17-month-old with significantly elevated compartment pressures successfully treated with antivenin and supportive care. A 17-month-old girl sustained a copperhead bite to the foot and presented with circumferential edema, erythema, and ecchymosis of the foot and distal ankle. The patient had palpable pulses and was neurologically intact. Four vials of Crotalidae polyvalent immune Fab was initiated and additional doses were administered in an attempt to achieve local control. Within 10 h of presentation, the patient's edema extended to the groin, although sensation was maintained and pulses were documented by Doppler. Lower-extremity compartment pressures were measured and were most notable for an anterior pressure of 85 mm Hg, despite having received 12 vials of antivenin. Fasciotomy was deferred and the patient received two additional six-vial doses of antivenin to achieve local control. Compartment pressures improved with a 2.2-cm mean decrease in limb diameter within 48 h. Maintenance dosing was initiated and the patient ultimately received a total of 26 vials of antivenin. The patient did not develop significant coagulopathy or thrombocytopenia. Swelling continued to improve with return of limb function. In this case, early and aggressive treatment with antivenin may have avoided invasive fasciotomy, and its use should be considered in patients with copperhead envenomation and significantly elevated compartment pressures. Copyright © 2014 Elsevier Inc. All rights reserved.

Bevacizumab-associated Sudden Onset of Multiple Monomorphic Comedones on the Scalp Successfully Treated with 30% Salicylic Acid Peels.

PubMed

Platsidaki, Eftychia; Balamoti, Evgenia; Kouris, Anargyros; Katsarou, Alexandra; Mylonakis, Nikolaos; Vavouli, Charitomeni; Kontochristopoulos, Georgios

2017-10-01

Bevacizumab is a humanized monoclonal antibody against vascular endothelial factor (VEGF) that targets tumor cell angiogenesis and proliferation. Although it is usually well tolerated, many side-effects have been reported. These include hypertension, bleeding, and thromboembolic events among others. Drug-associated cutaneous adverse effects are less common and include itching, exfoliative dermatitis, and acneiform eruptions. A man with bevacizumab-associated monomorphic skin eruption successfully was treated with 30% salicylic acid peels. To the author's knowledge, this is the first report of open comedones with no further inflammatory acne lesions that developed in a patient treated with bevacizumab. Complete remission of the rash was achieved after performing 30% salicylic peels, and the patient continued the chemotherapy as planned with no need of either dose reduction or discontinuation of bevacizumab.

Success and complications of an intra-ductal fully covered self-expanding metal stent (ID-FCSEMS) to treat anastomotic biliary strictures (AS) after orthotopic liver transplantation (OLT).

PubMed

Aepli, Patrick; St John, Andrew; Gupta, Saurabh; Hourigan, Luke F; Vaughan, Rhys; Efthymiou, Marios; Kaffes, Arthur

2017-04-01

Anastomotic biliary strictures (AS) after orthotopic liver transplantation (OLT) belong to the most common biliary complications and cause the biggest morbidity burden after OLT. Metal stents for benign biliary strictures are gaining acceptance with many published series. Traditional metal stent designs seem to have poor durability in AS after OLT. Novel intra-ductal stents are showing promise in these strictures. As a result, we designed a special stent with an antimigration waist and a short stent length with a long removal string that rests in the duodenum for easy removal. This is a retrospective multi-centre Australian study of AS after OLT treated with a novel intra-ductal fully covered self-expanding metal stent. From August 2008 to October 2014, records from three liver transplant centres were reviewed. Totally 36 ID-FCSEMS were inserted in 31 cases to treat an AS after OLT. The mean age of the patients was 56 years, and 61 % were male. The mean time of AS presentation after OLT was 20.3 months. Eight out of our 31 patients were previously treated using multiple plastic stenting over time without any success. Treatment with the ID-FCSEMS was performed with an average treatment time of 3.8 months. Stricture resolution was achieved in 100 %. All attempted stents removals were successful without any difficulty. Complications were reported in 6.5 %. It was pleasing that only one case of stent migration (2.8 %) was seen. Follow-up showed seven cases of AS recurrence (24.1 %), and all were treated successfully with repeat ERCP and stenting (some metal, some plastic). This novel ID-FCSEMS has a high clinical success and low complication rate, and in particular, there was only one case of stent migration. As a result, this stent type is preferred to traditional metal stents for treating AS after OLT.

Pyoderma gangrenosum, acne, and suppurative hidradenitis syndrome in end-stage renal disease successfully treated with adalimumab.

PubMed

De Wet, J; Jordaan, H F; Kannenberg, S M; Tod, B; Glanzmann, B; Visser, W I

2017-12-15

PASH syndrome (pyoderma gangrenosum, acne, and suppurative hidradenitis) forms part of the spectrum of autoinflammatory diseases. We report an unusual case of PASH syndrome in a patient with end-stagerenal disease (ESRD) who was successfully treated with the tumor necrosis factor inhibitor, adalimumab. The case underscores the challenges associatedwith the treatment of PASH syndrome as well as the ongoing search to establish a genetic basis for the syndrome. Renal impairment has been reported in association with pyoderma gangrenosum but has notbeen described in PASH syndrome. We believe this to be the first reported case of a patient who developed PASH syndrome in the setting of ESRD.

3-AP and Fludarabine in Treating Patients With Myeloproliferative Disorders, Chronic Myelomonocytic Leukemia, or Accelerated Phase or Blastic Phase Chronic Myelogenous Leukemia

ClinicalTrials.gov

2014-12-16

Accelerated Phase Chronic Myelogenous Leukemia; Atypical Chronic Myeloid Leukemia, BCR-ABL1 Negative; Blastic Phase Chronic Myelogenous Leukemia; Chronic Eosinophilic Leukemia; Chronic Myelomonocytic Leukemia; Essential Thrombocythemia; Philadelphia Chromosome Negative Chronic Myelogenous Leukemia; Polycythemia Vera; Primary Myelofibrosis; Relapsing Chronic Myelogenous Leukemia

Different immunophenotypical apoptotic profiles characterise megakaryocytes of essential thrombocythaemia and primary myelofibrosis.

PubMed

Florena, A M; Tripodo, C; Di Bernardo, A; Iannitto, E; Guarnotta, C; Porcasi, R; Ingrao, S; Abbadessa, V; Franco, V

2009-04-01

Essential thrombocythaemia (ET) and primary myelofibrosis (PMF) share some clinical and pathological features, but show different biological behaviour and prognosis. The latest contributions to understanding the nature of these disorders have focused on bone marrow microenvironment remodelling and proliferative stress, recognising megakaryocytes (MKCs) as "key-cells". The aim of this study was to investigate the apoptotic profile of ET and PMF MKCs in order to further characterise the biology of these disorders. Bone marrow biopsy samples from 30 patients with ET, and 30 patients with PMF, were immunophenotypically studied for the expression of pro-apoptotic (Fas, Fas-L, Bax, Bad) and anti-apoptotic (Bcl-2, Bcl-XL, hTERT (human telomerase reverse transcriptase)) molecules and the "executioner" molecule caspase-3. The fraction of MKCs undergoing apoptosis was assessed by deoxynucleotidyl transferase-mediated dUTP nick-end labelling. Only the mitochondrial pathway seemed to be involved in MKC apoptosis. The anti-apoptotic molecule Bcl-XL was predominantly found in ET MKCs (50.5% of ET MKCs versus 35% of PMF MKCs; p = 0.036), while pro-apoptotic molecules Bax and Bad showed a prevalent expression in PMF MKCs (30.5% of ET MKCs versus 55% of PMF MKCs; 41% of ET MKCs versus 52% of PMF MKCs; p = 0.001 and p = 0.068, respectively). A significant fraction of PMF MKCs were committed to apoptosis according to caspase-3 expression and TUNEL, while only few ET cells were committed to apoptosis. hTERT was significantly more expressed in PMF (32% of ET MKCs versus 46% of PMF MKCs; p = 0.022), in agreement with the proliferative nature of this disease. It was found that ET and PMF MKCs, which barely differ in terms of morphology and aggregation, are characterised by markedly different apoptotic profiles. The rather high apoptotic fraction of PMF was able to support the fibrotic nature of this process, while the anti-apoptotic profile of ET cells fits well with their "steady

Cirrhosis related chylous ascites successfully treated with TIPS.

PubMed

de Vries, G J; Ryan, B M; de Bièvre, M; Driessen, A; Stockbrugger, R W; Koek, G H

2005-04-01

We describe a patient with chylous ascites, who was extensively investigated for the cause. No malignant or lymphatic disease could be found, but a liver biopsy revealed liver cirrhosis. The chylous ascites was unsuccessfully treated with a sodium restriction diet, diuretics and a medium chain triglyceride diet. After the placement of a transjugular intrahepatic portosystemic shunt the ascites disappeared.

Overexpression of FKBP51 in idiopathic myelofibrosis regulates the growth factor independence of megakaryocyte progenitors.

PubMed

Giraudier, Stéphane; Chagraoui, Hédia; Komura, Emiko; Barnache, Stéphane; Blanchet, Benoit; LeCouedic, Jean Pierre; Smith, David F; Larbret, Frédéric; Taksin, Anne-Laure; Moreau-Gachelin, Françoise; Casadevall, Nicole; Tulliez, Michel; Hulin, Anne; Debili, Najet; Vainchenker, William

2002-10-15

Idiopathic myelofibrosis (IMF) is a chronic myeloproliferative disorder characterized by megakaryocyte hyperplasia and bone marrow fibrosis. Biologically, an autonomous megakaryocyte growth and differentiation is noticed, which contributes to the megakaryocyte accumulation. To better understand the molecular mechanisms involved in this spontaneous growth, we searched for genes differentially expressed between normal megakaryocytes requiring cytokines to grow and IMF spontaneously proliferating megakaryocytes. Using a differential display technique, we found that the immunophilin FKBP51 was 2 to 8 times overexpressed in megakaryocytes derived from patients' CD34(+) cells in comparison to normal megakaryocytes. Overexpression was moderate and confirmed in 8 of 10 patients, both at the mRNA and protein levels. Overexpression of FKBP51 in a UT-7/Mpl cell line and in normal CD34(+) cells induced a resistance to apoptosis mediated by cytokine deprivation with no effect on proliferation. FKBP51 interacts with both calcineurin and heat shock protein (HSP)70/HSP90. However, a mutant FKBP51 deleted in the HSP70/HSP90 binding site kept the antiapoptotic effect, suggesting that the calcineurin pathway was responsible for the FKBP51 effect. Overexpression of FKBP51 in UT-7/Mpl cells induced a marked inhibition of calcineurin activity. Pharmacologic inhibition of calcineurin by cyclosporin A mimicked the effect of FKBP51. The data support the conclusion that FKBP51 inhibits apoptosis through a calcineurin-dependent pathway. In conclusion, FKBP51 is overexpressed in IMF megakaryocytes and this overexpression could be, in part, responsible for the megakaryocytic accumulation observed in this disorder by regulating their apoptotic program.

[Experitoneal bladder perforation due to in-dwelling urethral catheter successfully treated by urethral drainage: a case report].

PubMed

Okuda, Hidenobu; Tei, Norihide; Shimizu, Kiyonori; Imazu, Tetsuo; Yoshimura, Kazuhiro; Kiyohara, Hisakazu

2008-07-01

Perforation of the bladder related to long-term indwelling urethral catheter is a rare and serious complication. A 85-year-old man with an indwelling urethral catheter presented severe hematuria, abdominal pain with rebound tenderness and muscular tension over the suprapubic area after the exchange of the urethral catheter. Computed tomography and cystogram revealed experitoneal bladder perforation due to indwelling catheter. Three weeks after the indwelling urethral catheter had been placed, the perforation was closed. In most cases, laparotomy and suprapubic cystostomy are performed. We describe the case of experitoneal bladder perforation successfully treated by urethral drainage.

Screening and monitoring of MPL W515L mutation with real-time PCR in patients with myelofibrosis undergoing allogeneic-SCT.

PubMed

Alchalby, H; Badbaran, A; Bock, O; Fehse, B; Bacher, U; Zander, A R; Kröger, N

2010-09-01

Monitoring of minimal residual disease (MRD) after allogeneic (allo)-SCT for myelofibrosis (MF) allows recognizing the depth of remission and thus guides application of appropriate therapeutic interventions. MPL W515L/K mutations, which are detected in 5-10% of JAK2V617F-negative patients, may be useful for this purpose. Using a highly sensitive quantitative PCR method, we tested 90 patients with MF who underwent allo-SCT for the presence of MPL W515L/K mutations. Two patients with primary MF were found to harbor MPLW515L while no patient was positive for MPLW515K mutation. Both patients were JAK2V617F negative and cleared the mutation rapidly after allo-SCT and remained negative for a median follow-up of 19 months. The results of molecular monitoring correlated well with other remission parameters such as normalization of peripheral blood counts and morphology and complete donor chimerism. We conclude that MPLW515L can be cleared after allo-SCT and hence may be used as an MRD marker in a proportion of JAK2V617F-negative MF patients.

Chronic active Epstein-Barr virus infection (CAEBV) successfully treated with allogeneic peripheral blood stem cell transplantation.

PubMed

Taketani, T; Kikuchi, A; Inatomi, J; Hanada, R; Kawaguchi, H; Ida, K; Oh-Ishi, T; Arai, T; Kishimoto, H; Yamamoto, K

2002-03-01

We report a pediatric case of CAEBV and T cell-based Hodgkin's-like disease successfully treated with allo PBSCT from an HLA-matched sibling. The diagnosis of CAEBV was made from clinical signs and the presence of the EBV genome in PBMC and tumor cells. Conditioning with busulfan (BU) + etoposide (VP16) + cyclophosphamide (CY) was effective and well tolerated. EBV was totally eradicated by 3 months after allo PBSCT. Although she suffered from chronic GVHD of the liver, she has been well and free of disease for 47 months since PBSCT. We suggest allo PBSCT for CAEBV as a potent therapeutic strategy for eradication of the EBV genome and allowing immunological reconstitution.

A case of reccuring giant condyloma of vulva in infant without sexual abuse successfully treated with electrocoagulation in Benin.

PubMed

Akpadjan, Fabrice; Adégbidi, Hugues; Attinsounon, Cossi Angelo; Koudoukpo, Christiane; Dégboé, Bérénice; Agbessi, Nadège; Atadokpèdé, Félix

2017-01-01

We report here a case of giant vulval condyloma in a two-year-old infant infected by her "baby sitter" without sexual abuse. Treated by surgical excision coupled with electrocoagulation, it was noted a rapid recurrence two weeks after treatment requiring a second electrocoagulation session. More than a year later, no lesion was noted, thus demonstrating therapeutic success. The unavailability of imiquimod in our context requires a systematic use of invasive treatment regardless of the age of the patient.

Clonal evolution revealed by whole genome sequencing in a case of primary myelofibrosis transformed to secondary acute myeloid leukemia.

PubMed

Engle, E K; Fisher, D A C; Miller, C A; McLellan, M D; Fulton, R S; Moore, D M; Wilson, R K; Ley, T J; Oh, S T

2015-04-01

Clonal architecture in myeloproliferative neoplasms (MPNs) is poorly understood. Here we report genomic analyses of a patient with primary myelofibrosis (PMF) transformed to secondary acute myeloid leukemia (sAML). Whole genome sequencing (WGS) was performed on PMF and sAML diagnosis samples, with skin included as a germline surrogate. Deep sequencing validation was performed on the WGS samples and an additional sample obtained during sAML remission/relapsed PMF. Clustering analysis of 649 validated somatic single-nucleotide variants revealed four distinct clonal groups, each including putative driver mutations. The first group (including JAK2 and U2AF1), representing the founding clone, included mutations with high frequency at all three disease stages. The second clonal group (including MYB) was present only in PMF, suggesting the presence of a clone that was dispensable for transformation. The third group (including ASXL1) contained mutations with low frequency in PMF and high frequency in subsequent samples, indicating evolution of the dominant clone with disease progression. The fourth clonal group (including IDH1 and RUNX1) was acquired at sAML transformation and was predominantly absent at sAML remission/relapsed PMF. Taken together, these findings illustrate the complex clonal dynamics associated with disease evolution in MPNs and sAML.

Cotard's syndrome with schizophreniform disorder can be successfully treated with electroconvulsive therapy: case report

PubMed Central

Caliyurt, Okan; Vardar, Erdal; Tuglu, Cengiz

2004-01-01

We report a case of Cotard's syndrome associated with psychotic symptoms. A 27-year-old man was admitted to hospital with the diagnosis of schizophreniform disorder. His presenting symptoms, which had started 1 month before hospital admission, were somatic delusions of gastrointestinal and cardiovascular malfunction and the absence of a stomach, which resulted in a decrease in weight from 75 kg to 63 kg in 1 month. Cranial computed tomographic images showed dilatation of the lateral and third ventricles, whereas magnetic resonance imaging revealed central atrophy and lateral ventricle dilatation. Single- photon emission computed tomography demonstrated left temporal, left frontal and left parietal hypoperfusion. The patient did not respond to antipsychotic therapies, but he was successfully treated with electroconvulsive therapy. This report emphasizes that Cotard's syndrome may be accompanied by lesions of the left hemisphere and that electroconvulsive therapy could be the first-line therapy in such patients with psychotic disorder. PMID:15069468

A case of reccuring giant condyloma of vulva in infant without sexual abuse successfully treated with electrocoagulation in Benin

PubMed Central

Akpadjan, Fabrice; Adégbidi, Hugues; Attinsounon, Cossi Angelo; Koudoukpo, Christiane; Dégboé, Bérénice; Agbessi, Nadège; Atadokpèdé, Félix

2017-01-01

We report here a case of giant vulval condyloma in a two-year-old infant infected by her “baby sitter” without sexual abuse. Treated by surgical excision coupled with electrocoagulation, it was noted a rapid recurrence two weeks after treatment requiring a second electrocoagulation session. More than a year later, no lesion was noted, thus demonstrating therapeutic success. The unavailability of imiquimod in our context requires a systematic use of invasive treatment regardless of the age of the patient. PMID:28904687

Methotrexate-treated ectopic pregnancy: beta human chorionic gonadotropin serum changes as a success predictor using a mathematical model validation.

PubMed

Kovaleva, Aleksandra; Irishina, Natalia; Pereira, Augusto; Cuesta-Guardiola, Tatiana; Ortiz-Quintana, Luis

2017-03-01

Surgical rescue of methotrexate-treated ectopic pregnancy is necessary when tubal rupture or medical therapy failure is detected during post-therapeutic monitoring. It is known that an increased beta human chorionic gonadotropin (β-hCG) concentration is the most important factor associated with treatment failure. Therefore, we suggested that relative changes in serum β-hCG could predict a successful result of medical treatment, leading to facilitation of the decision to forgo the prospect of possible surgical rescue. A retrospective observational study of 115 patients with an ectopic pregnancy who were treated with a single dosage protocol of 50mg/m 2 of methotrexate injected intramuscularly was performed at Puerta de Hierro University Hospital and Gregorio Marañón University General Hospital. Standard statistical tests were applied in order to evaluate the relative changes in β-hCG concentration between the 1st and the 4th days following methotrexate injection. Methotrexate treatment has a 95% probability to be successful if the relative change of β-hCG from the 1st to the 4th day of monitoring is within the following interval: [-1.02; 0.15]. Moreover, if the values of β-hCG-relative change from 1st to 4th day of monitoring are within [0.54; 1.2], it assures a negative result of treatment with 95% probability. Therefore, the value 0.15 (15%) of β-hCG relative change can be considered a cut-off value for a positive result to treatment. Our data support that negative β-hCG relative changes on the 4th day of treatment likely predict a successful result of methotrexate therapy, with a cut-off point of 0.15. Expectant management should be carried out in these cases if no clinical indications of surgery are presented. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Acute chloroform ingestion successfully treated with intravenously administered N-acetylcysteine.

PubMed

Dell'Aglio, Damon M; Sutter, Mark E; Schwartz, Michael D; Koch, David D; Algren, D A; Morgan, Brent W

2010-06-01

Chloroform, a halogenated hydrocarbon, causes central nervous system depression, cardiac arrhythmias, and hepatotoxicity. We describe a case of chloroform ingestion with a confirmatory serum level and resultant hepatotoxicity successfully treated with intravenously administered N-acetylcysteine (NAC). A 19-year-old man attempting suicide ingested approximately 75 mL of chloroform. He was unresponsive and intubated upon arrival. Intravenously administered NAC was started after initial stabilization was complete. His vital signs were normal. Admission laboratory values revealed normal serum electrolytes, AST, ALT, PT, BUN, creatinine, and bilirubin. Serum ethanol level was 15 mg/dL, and aspirin and acetaminophen were undetectable. The patient was extubated but developed liver function abnormalities with a peak AST of 224 IU/L, ALT of 583 IU/L, and bilirubin level reaching 16.3 mg/dL. NAC was continued through hospital day 6. Serum chloroform level obtained on admission was 91 μg/mL. The patient was discharged to psychiatry without known sequelae and normal liver function tests. The average serum chloroform level in fatal cases of inhalational chloroform poisoning was 64 μg/mL, significantly lower than our patient. The toxicity is believed to be similar in both inhalation and ingestion routes of exposure, with mortality predominantly resulting from anoxia secondary to central nervous system depression. Hepatocellular toxicity is thought to result from free radical-induced oxidative damage. Previous reports describe survival after treatment with orally administered NAC, we report the first use of intravenously administered NAC for chloroform ingestion. Acute oral ingestion of chloroform is extremely rare. Our case illustrates that with appropriate supportive care, patients can recover from chloroform ingestion, and intravenously administered NAC may be of benefit in such cases.

Primary myelofibrosis with or without mutant MPL: comparison of survival and clinical features involving 603 patients.

PubMed

Pardanani, A; Guglielmelli, P; Lasho, T L; Pancrazzi, A; Finke, C M; Vannucchi, A M; Tefferi, A

2011-12-01

MPL and JAK2V617F mutation analysis was performed in 603 patients with primary myelofibrosis (PMF) seen at the Mayo Clinic, USA (n=329) or University of Florence, Italy (n=274). Mutant MPL was detected in 49 (8.1%) patients and JAK2V617F in 350 (58%); 4 patients showed both mutations. MPLW515L/K was the commonest mutation; 2 patients showed novel mutations (L513ins and Q516-P518insAAAA). The US and Italy patient cohorts were separately analyzed for comparison of survival and clinical features between MPL-mutated, JAK2-mutated and JAK2/MPL-unmutated cases. JAK2/MPL-unmutated patients were significantly younger than their JAK2-mutated counterparts, in both patient cohorts (P<0.01). In the Florence only cohort, the presence of mutant MPL was associated with older age (P<0.01) and constitutional symptoms (P=0.04) and JAK2V617F with higher hemoglobin (P<0.01) and leukocyte (P=0.03) count; neither patient cohort showed significant associations with platelet count, hemoglobin <10 g/dl, abnormal/unfavorable karyotype, spleen size or prognostic score distribution. To date, 240 deaths and 79 leukemic transformations have been documented among all 603 study patients. Multivariable analysis disclosed no significant difference in overall or leukemia-free survival between the three molecular subgroups. We conclude that the presence of mutant MPL has narrow and inconsistent phenotypic effect in PMF and does not influence overall or leukemia-free survival.

Extubation success in premature infants with respiratory distress syndrome treated with bi-level nasal continuous positive airway pressure versus nasal intermittent positive pressure ventilation.

PubMed

Thomas, Patricia E; LeFlore, Judy

2013-01-01

Infants born prematurely with respiratory distress syndrome are at high risk for complications from mechanical ventilation. Strategies are needed to minimize their days on the ventilator. The purpose of this study was to compare extubation success rates in infants treated with 2 different types of continuous positive airway pressure devices. A retrospective cohort study design was used. Data were retrieved from electronic medical records for patients in a large, metropolitan, level III neonatal intensive care unit. A sample of 194 premature infants with respiratory distress syndrome was selected, 124 of whom were treated with nasal intermittent positive pressure ventilation and 70 with bi-level variable flow nasal continuous positive airway pressure (bi-level nasal continuous positive airway pressure). Infants in both groups had high extubation success rates (79% of nasal intermittent positive pressure ventilation group and 77% of bi-level nasal continuous positive airway pressure group). Although infants in the bi-level nasal continuous positive airway pressure group were extubated sooner, there was no difference in duration of oxygen therapy between the 2 groups. Promoting early extubation and extubation success is a vital strategy to reduce complications of mechanical ventilation that adversely affect premature infants with respiratory distress syndrome.

JAK2 V617F, MPL W515L and JAK2 Exon 12 Mutations in Chinese Patients with Primary Myelofibrosis.

PubMed

Xia, Jun; Lu, Mi-Ze; Jiang, Yuan-Qiang; Yang, Guo-Hua; Zhuang, Yun; Sun, Hong-Li; Shen, Yun-Feng

2012-03-01

JAK2 V617F, MPL W515L and JAK2 exon 12 mutations are novel acquired mutations that induce constitutive cytokine-independent activation of the JAK-STAT pathway in myeloproliferative disorders (MPD). The discovery of these mutations provides novel mechanism for activation of signal transduction in hematopoietic malignancies. This research was to investigate their prevalence in Chinese patients with primary myelofibrosis (PMF). We introduced allele-specific PCR (AS-PCR) combined with sequence analysis to simultaneously screen JAK2 V617F, MPL W515L and JAK2 exon 12 mutations in 30 patients with PMF. Fifteen PMF patients (50.0%) carried JAK2 V617F mutation, and only two JAK2 V617F-negative patients (6.7%) harbored MPL W515L mutation. None had JAK2 exon 12 mutations. Furthermore, these three mutations were not detected in 50 healthy controls. MPL W515L and JAK2 V617F mutations existed in PMF patients but JAK2 exon 12 mutations not. JAK2 V617F and MPL W515L and mutations might contribute to the primary molecular pathogenesis in patients with PMF.

Microbial succession in a compost-packed biofilter treating benzene-contaminated air.

PubMed

Borin, Sara; Marzorati, Massimo; Brusetti, Lorenzo; Zilli, Mario; Cherif, Hanene; Hassen, Abdennaceur; Converti, Attilio; Sorlini, Claudia; Daffonchio, Daniele

2006-03-01

Air artificially contaminated with increasing concentrations of benzene was treated in a laboratory scale compost-packed biofilter for 240 days with a removal efficiency of 81-100%. The bacterial community in the packing material (PM) at different heights of the biofilter was analysed every 60 days. Bacterial plate counts and ribosomal intergenic spacer analysis (RISA) of the isolated strains showed that the number of cultivable aerobic heterotrophic bacteria and the species diversity increased with benzene availability. Identification of the isolated species and the main bands in denaturing gradient gel electrophoresis (DGGE) profiles from total compost DNA during the treatment revealed that, at a relatively low volumetric benzene load (1.2< or =VBL< or =6.4 g m(-3) (PM) h(-1)), besides low G+C Gram positive bacteria, originally present in the packing compost, bacteroidetes and beta- and gamma-proteobacteria became detectable in the colonising population. At the VBL value (24.8 g m(-3) (PM) h(-1)) ensuring the maximum elimination capacity of the biofilter (20.1 g m(-3) (PM) h(-1)), strains affiliated to the genus Rhodococcus dominated the microflora, followed by beta-proteobacteria comprising the genera Bordetella and Neisseria. Under these conditions, more than 35% of the isolated strains were able to grow on benzene as the sole carbon source. Comparison of DGGE and automated RISA profiles of the total community and isolated strains showed that a complex bacterial succession occurred in the reactor in response to the increasing concentrations of the pollutant and that cultivable bacteria played a major role in benzene degradation under the adopted conditions.

Analysis of Class II patients, successfully treated with the straight-wire and Forsus appliances, based on cervical vertebral maturation status.

PubMed

Servello, David F; Fallis, Drew W; Alvetro, Lisa

2015-01-01

To assess skeletal and dental changes in patients successfully treated with the Forsus appliance based on cervical vertebral maturation status. Forty-seven Class II patients, successfully treated with the Forsus appliance, were divided into peak and postpeak growth groups determined immediately prior to Forsus placement. The mean (SD) ages of the peak and postpeak groups were 13.4 (1.0) and 14.1 (1.3) years, respectively. Superimpositions of initial, Forsus placement, Forsus removal, and final cephalometric radiographs were completed, allowing the measurement of changes during three treatment phases. There were no significant differences between groups during treatment phase 1 (alignment/leveling), with both groups demonstrating a worsening of the Class II molar relationship. However, during treatment phase 2 (Class II correction), patients within the peak group demonstrated significantly higher mean apical base, mandibular and molar changes, and an increased rate of change compared with those in the postpeak group. No significant differences were observed during treatment phase 3 (detail/finishing). Following an initial worsening of the Class II molar relationship as a result of straight-wire appliance effects, Forsus appliance treatment initiated during cervical vertebral maturation status (CS) 3-4 elicits more effective and efficient correction of Class II molar relationships than when initiated during CS 5-6. Data support that these effects are due mainly to maxillary skeletal and dentoalveolar restraint during a period of more rapid mandibular growth.

Essential thrombocythemia in young individuals: frequency and risk factors for vascular events and evolution to myelofibrosis in 126 patients.

PubMed

Alvarez-Larrán, A; Cervantes, F; Bellosillo, B; Giralt, M; Juliá, A; Hernández-Boluda, J C; Bosch, A; Hernández-Nieto, L; Clapés, V; Burgaleta, C; Salvador, C; Arellano-Rodrigo, E; Colomer, D; Besses, C

2007-06-01

The frequency of vascular events and evolution to myelofibrosis (MF) in young individuals with essential thrombocythemia (ET) is not well known. The incidence and predisposing factors to such complications was studied in 126 subjects diagnosed with ET at a median age of 31 years (range: 5-40). Overall survival and probability of survival free of thrombosis, bleeding and MF were analyzed by the Kaplan-Meier method and the presence of the Janus Kinase 2 (JAK2) V617F mutation correlated with the appearance of such complications. The JAK2 mutation (present in 43% of patients) was associated with higher hemoglobin (Hb) (P<0.001) and lower platelets at diagnosis. With a median follow-up of 10 years (range: 4-25), 31 thrombotic events were registered (incidence rate: 2.2 thromboses/100 patients/year). When compared with the general population, young ET patients showed a significant increase in stroke (odds ratio 50, 95% CI: 21.5-115) and venous thromboses (odds ratio 5.3, 95% CI: 3.9-10.6). Thrombosis-free survival was 84% at 10 years, with tobacco use being associated with higher risk of thrombosis. Actuarial freedom from evolution to MF was 97% at 10 years. In conclusion, young ET patients have thrombotic events, especially stroke and venous thrombosis, more frequently than generally considered, whereas they rarely transform to MF.

Attenuation of cue-induced smoking urges and brain reward activity in smokers treated successfully with bupropion.

PubMed

Weinstein, A; Greif, J; Yemini, Z; Lerman, H; Weizman, A; Even-Sapir, E

2010-06-01

Twenty-two regular smokers (15+ cigarettes per day) were treated with bupropion and group therapy for 2 months. Subjects underwent positron emission tomography (PET) studies using measures of brain global and regional glucose metabolism (regional cerebral metabolic rates of glucose [rCMRglc]) with [18F]-Fluorodeoxyglucose (FDG) twice, after watching a videotape showing smoking scenes and after watching a control movie in counter-balanced order. A questionnaire of smoking urges (QSU) was filled in before and after watching both the movies. Changes in brain metabolic rates of FDG were analysed using Statistical Parametric Maps (SPM 2) in 11 smokers who abstained from smoking in comparison with 11 smokers who continued to smoke during the second month of treatment. Still-smokers had higher craving scores after watching the videotape showing smoking scenes compared with non-smokers. Second, watching the videotape showing smoking scenes compared with the control videotape in still-smokers resulted in increased metabolic rates in the striatum, thalamus and midbrain. Third, the ratings of the urge to smoke cigarettes while watching the videotape showing smoking scenes in still-smokers were associated with brain metabolic activity in the ventral striatum, anterior cingulate, orbitofrontal cortex, middle temporal lobe, hippocampus, insula, midbrain and thalamus. In conclusion, successfully treated smokers showed attenuated craving and reduced activity in the mesolimbic reward circuit.

Lung abscess combined with chronic osteomyelitis of the mandible successfully treated with video-assisted thoracoscopic surgery.

PubMed

Arai, Hiromasa; Inui, Kenji; Watanabe, Keisuke; Watanuki, Kei; Okudela, Koji; Tsuboi, Masahiro; Masuda, Munetaka

2015-04-01

With the progress of antibiotic therapy, the mortality of lung abscess has been improved, and surgical intervention has declined. However, surgery is still required in selected cases that are intractable to antibiotic treatment. Video-assisted thoracoscopic surgery (VATS) is beneficial for treatment and/or diagnosis of pulmonary disease as it provides a less invasive surgical technique and reduces prolongation of post-operative recovery. However, the indication of VATS lobectomy for lung abscess is controversial as a result of particular complications, i.e. wet lung, intrapleural adhesion and ease of bleeding. We herein report a rare combination of lung abscess and osteomyelitis of mandible resulting from the same pathogen successfully treated with VATS lobectomy. We propose VATS lobectomy for lung abscess. This procedure might be the best treatment candidate for selected cases of lung abscess. © 2014 John Wiley & Sons Ltd.

A data-driven network model of primary myelofibrosis: transcriptional and post-transcriptional alterations in CD34+ cells.

PubMed

Calura, E; Pizzini, S; Bisognin, A; Coppe, A; Sales, G; Gaffo, E; Fanelli, T; Mannarelli, C; Zini, R; Norfo, R; Pennucci, V; Manfredini, R; Romualdi, C; Guglielmelli, P; Vannucchi, A M; Bortoluzzi, S

2016-06-24

microRNAs (miRNAs) are relevant in the pathogenesis of primary myelofibrosis (PMF) but our understanding is limited to specific target genes and the overall systemic scenario islacking. By both knowledge-based and ab initio approaches for comparative analysis of CD34+ cells of PMF patients and healthy controls, we identified the deregulated pathways involving miRNAs and genes and new transcriptional and post-transcriptional regulatory circuits in PMF cells. These converge in a unique and integrated cellular process, in which the role of specific miRNAs is to wire, co-regulate and allow a fine crosstalk between the involved processes. The PMF pathway includes Akt signaling, linked to Rho GTPases, CDC42, PLD2, PTEN crosstalk with the hypoxia response and Calcium-linked cellular processes connected to cyclic AMP signaling. Nested on the depicted transcriptional scenario, predicted circuits are reported, opening new hypotheses. Links between miRNAs (miR-106a-5p, miR-20b-5p, miR-20a-5p, miR-17-5p, miR-19b-3p and let-7d-5p) and key transcription factors (MYCN, ATF, CEBPA, REL, IRF and FOXJ2) and their common target genes tantalizingly suggest new path to approach the disease. The study provides a global overview of transcriptional and post-transcriptional deregulations in PMF, and, unifying consolidated and predicted data, could be helpful to identify new combinatorial therapeutic strategy. Interactive PMF network model: http://compgen.bio.unipd.it/pmf-net/.

A WORKABLE DEFINITION OF SUCCESS

EPA Science Inventory

The most desirable definition of success from the point of view of environmental stewardship is to treat the entire plume to MCLs. Technically, has proven impossible to attain at many sites. A less desirable definition of success is to contain the source of contamination and tr...

Successfully treated necrotizing fasciitis using extracorporeal life support combined with hemoadsorption device and continuous renal replacement therapy.

PubMed

Eid, Maroua; Fouquet, Olivier; Darreau, Cédric; Pierrot, Marc; Kouatchet, Achille; Mercat, Alain; Baufreton, Christophe

2018-03-01

Necrotizing fasciitis represents a life-threatening infectious condition that causes spreading necrotisis of superficial fascia and subcutaneous cellular tissues. We describe the case of a patient diagnosed with septic and toxic shocks leading to multiple organ failure successfully treated with a combination of extracorporeal life support, continuous renal replacement therapy, and a hemoadsorption device. A 41-year-old patient presented with necrotizing fasciitis and multi-organ failure. Initial extracorporeal life support therapy was implanted, compensating for systolic failure. Due to acute renal failure that persisted in time, continuous renal replacement therapy was added. Despite these treatments and as a last attempt to control the septic condition, a CytoSorb ® hemoadsorption device was installed in parallel to the extracorporeal life support circuit and two sessions were run. During the days following CytoSorb ® treatment, hemodynamic stabilization was observed, as well as normalization of lactic acidosis and blood parameters. This case describes the successful use of CytoSorb ® with continuous renal replacement therapy and extracorporeal life support in a combined way to overcome a critical phase of septic shock in a young adult patient. This combination of treatments turned out to be efficient for this patient in the context of necrotizing fasciitis.

Non-Thyroidal Illness Syndrome in Patients Exposed to Indoor Air Dampness Microbiota Treated Successfully with Triiodothyronine.

PubMed

Somppi, Taija Liisa

2017-01-01

Long-term exposure to dampness microbiota induces multi-organ morbidity. One of the symptoms related to this disorder is non-thyroidal illness syndrome (NTIS). A retrospective study was carried out in nine patients with a history of mold exposure, experiencing chronic fatigue, cognitive disorder, and different kinds of hypothyroid symptoms despite provision of levothyroxine (3,5,3',5'-tetraiodothyronine, LT4) monotherapy. Exposure to volatile organic compounds present in water-damaged buildings including metabolic products of toxigenic fungi and mold-derived inflammatory agents can lead to a deficiency or imbalance of many hormones, such as active T3 hormone. Since the 1970s, the synthetic prohormone, levothyroxine (LT4), has been the most commonly prescribed thyroid hormone in replacement monotherapy. It has been presumed that the peripheral conversion of T4 (3,5,3',5'-tetraiodothyronine) into T3 (3,5,3'-triiodothyronine) is sufficient to satisfy the overall tissue requirements. However, evidence is presented that this not the case for all patients, especially those exposed to indoor air molds. This retrospective study describes the successful treatment of nine patients in whom NTIS was treated with T3-based thyroid hormone. The treatment was based on careful interview, clinical monitoring, and laboratory analysis of serum free T3 (FT3), reverse T3 (rT3) and thyroid-stimulating hormone, free T4, cortisol, and dehydroepiandrosterone (DHEA) values. The ratio of FT3/rT3 was calculated. In addition, some patients received adrenal support with hydrocortisone and DHEA. All patients received nutritional supplementation and dietary instructions. During the therapy, all nine patients reported improvements in all of the symptom groups. Those who had residual symptoms during T3-based therapy remained exposed to indoor air molds in their work places. Four patients were unable to work and had been on disability leave for a long time during LT4 monotherapy. However, during the

Chylous ascites occurring after low anterior resection of the rectum successfully treated with an oral fat-free elemental diet (Elental(®)).

PubMed

Nakayama, Gakuryu; Morioka, Daisuke; Murakami, Takashi; Takakura, Hideki; Miura, Yasuhiko; Togo, Shinji

2012-06-01

Chylous ascites occurring after abdominal surgery is rare. Despite being potentially critical, there is no definite treatment guideline because of its rarity. Here we present a case of massive chylous ascites occurring after rectal surgery which was successfully treated with an oral fat-free elemental diet (ED). A 67-year-old man underwent low anterior resection with para-aortic lymphadenectomy for advanced rectal cancer. Early postoperative course was uneventful and the patient was discharged from hospital 10 days after surgery; however, after discharge, abdominal distension rapidly developed. Abdominal computed tomography (CT) performed 3 weeks after surgery revealed massive ascites and laboratory findings showed remarkable hypoproteinemia and lymphopenia. Urgent diagnostic paracentesis showed the ascites to be a white milky fluid containing high levels of triglycerides (564 mg/dl), leading to a diagnosis of chyloperitoneum. Daily nutrition of the patient was entirely with a fat-free ED (30 kcal/kg/day of Elental(®), Ajinomoto Pharmaceutical Co. Ltd, Tokyo, Japan). After the initiation of oral Elental(®), abdominal distension, hypoproteinemia, and lymphopenia gradually improved. Abdominal CT performed 7 weeks after surgery showed no ascitic fluid in the abdomen, and thereafter a normal diet was initiated. Since then, no relapse of chyloperitoneum has been proven. As a result, the chylous ascites was successfully treated in the outpatient clinic.

[Three cases of the malignant esophageal stenosis successfully treated with the Niti-S™ esophageal stent].

PubMed

Isohata, Noriyuki; Naritaka, Yoshihiko; Asaka, Shinichi; Shimakawa, Takeshi; Miyaki, Akira; Yamaguchi, Kentaro; Murayama, Minoru; Katsube, Takao; Ogawa, Kenji

2011-11-01

We herein report three cases of the malignant esophageal stenosis successfully treated with the Niti-S™ esophageal stent. CASE 1: The hilar lung cancer and its mediastinal lymph node metastasis pressed the esophagus extramurally and caused the marked stenosis. CASE 2: A metastatic lymph node along the left laryngeal nerve caused the stenosis of the trachea. A primary esophageal lesion located at the middle thoracic esophagus also caused the marked stenosis. At first, tracheal stent was placed because of dyspnea, and two weeks later, we placed an esophageal stent. Case 3: Esophageal cancer at lower thoracic esophagus after definitive radiation therapy caused the marked stenosis. Because of the stenosis of esophago-gastric junction( EGJ), we used an esophageal stent with a long cover in order to prevent a reflux into the esophagus. This new Niti-STM esophageal stent was easy to place at the stenosis without difficulty using a conventional device. The symptom was improved immediately for each case. We hope this new device will be used widely.

Thrombosis in essential thrombocytemia and early/prefibrotic primary myelofibrosis: the role of the WHO histological diagnosis.

PubMed

Rupoli, Serena; Goteri, Gaia; Picardi, Paola; Micucci, Giorgia; Canafoglia, Lucia; Scortechini, Anna Rita; Federici, Irene; Giantomassi, Federica; Da Lio, Lidia; Zizzi, Antonio; Honorati, Elisa; Leoni, Pietro

2015-04-16

Vascular events represent the most frequent complications of thrombocytemias. We aimed to evaluate their risk in the WHO histologic categories of Essential Thrombocytemia (ET) and early Primary Myelofibrosis (PMF). From our clinical database of 283 thrombocytemic patients, we selected those with available bone marrow histology performed before any treatment, at or within 1 year from diagnosis, and reclassified the 131 cases as true ET or early PMF, with or without fibrosis, according to the WHO histological criteria. Vaso-occlusive events at diagnosis and in the follow-up were compared in the WHO-groups. Histologic review reclassified 61 cases as ET and 72 cases as early PMF (26 prefibrotic and 42 with grade 1 or 2 fibrosis). Compared to ET, early PMF showed a significant higher rate of thrombosis both in the past history (22% vs 8%) and at diagnosis (15.2% vs 1.6%), and an increased leukocyte count (8389 vs 7500/mmc). Venous thromboses (mainly atypical) were relatively more common in PMF than in ET. Patients with prefibrotic PMF, although younger, showed a significant higher 15-year risk of developing thrombosis (48% vs 16% in fibrotic PMF and 17% in ET). At multivariate analysis, age and WHO histology were both independent risk-factors for thrombosis during follow-up; patients >60 yr-old or with prefibrotic PMF showed a significantly higher risk at 20 years than patients <60 yr-old with ET or fibrotic PMF (47% vs 4%, p = 0.005). Our study support the importance of WHO histologic categories in the thrombotic risk stratification of patients with thrombocytemias. The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2020211863144412 .

Correction of the Abnormal Trafficking of Primary Myelofibrosis CD34+ Cells by Treatment with Chromatin Modifying Agents

PubMed Central

Wang, Xiaoli; Zhang, Wei; Ishii, Takefumi; Sozer, Selcuk; Wang, Jiapeng; Xu, Mingjiang; Hoffman, Ronald

2011-01-01

The abnormal trafficking of CD34+ cells is a unique characteristic of primary myelofibrosis (PMF). We have further studied the behavior of PMF CD34+ cells by examining their homing to the marrow and the spleens of NOD/SCID mice. Following the infusion of PMF and normal G-CSF mobilized peripheral blood (mPB) CD34+ cells into NOD/SCID mice, reduced numbers of PMF CD34+ cells and CFU-GM as compared to mPB were detected in the marrow of these mice while similar numbers of PMF and mPB CD34+ cells and CFU-GM homed to their spleens. The abnormal homing of PMF CD34+ cells was associated with reduced expression of CXCR4, but was not related to the presence of JAK2V617F. The sequential treatment of PMF CD34+ cell with the chromatin modifying agents, 5-aza-2'-deoxycytidine (5azaD) and trichostatin A (TSA) but not treatment with small molecule inhibitors of JAK2 resulted in the generation of increased numbers of CD34+CXCR4+ cells which was accompanied by enhanced homing of PMF CD34+ cells to the marrow but not the spleens of NOD/SCID mice. Following 5azaD/TSA treatment JAK2V617F negative PMF hematopoietic progenitor cells preferentially homed to the marrow but not the spleens of recipient mice. Our data suggest that PMF CD34+ cells are characterized by a reduced ability to home to the marrow but not the spleens of NOD/SCID mice and that this homing defect can be corrected by sequential treatment with chromatin modifying agents. PMID:19752087

Prolonged catatonic stupor successfully treated with aripiprazole in an adolescent male with schizophrenia: a case report.

PubMed

Kirino, Eiji

2010-10-01

We present the case of a fifteen-year-old adolescent male with schizophrenia who had long-term catatonic stupor and was successfully treated with aripiprazole. The onset of his stupor manifested rapidly after experiencing prodromal symptoms for two months. He was left untreated without adequate food ingestion for three weeks because of his parents' religious faith, and was severely dehydrated and malnourished upon admission to our hospital. After his physical recovery, treatment with risperidone (0.5-2.0 mg, 5 weeks) was started. However, hypersedation occurred, and the risperidone was switched to aripiprazole, with dose increases up to 18 mg/day (5 months). As a result, he recovered from his totally noncommunicative state. Aripiprazole, which has a unique pharmacological mechanism of action distinct from other atypical antipsychotics and an excellent safety profile, may be effective in the treatment of some schizophrenic patients with stupor, which sometimes carries a risk of physical debilitation and requires special attention due to the risk of adverse drug reactions.

[A case of pancreatic and duodenal fistula after total gastrectomy successfully treated with coagulation factor XIII].

PubMed

Nishino, Hitoe; Kojima, Kazuhiro; Oshima, Hirokazu; Nakagawa, Koji; Fumura, Masao; Kikuchi, Norio

2013-11-01

Pancreatic fistula( PF) is a challenging postoperative complication. We report a case of PF following gastrectomy successfully treated using intravenous coagulation factor XIII( FXIII).A 78-year-old man with early gastric cancer underwent total gastrectomy with Roux-en-Y reconstruction. PF developed postoperatively, following which, leakage from the duodenal stump was observed. Percutaneous drainage and re-operative surgery were performed. A somatostatin analogue, antibiotic drugs, and gabexate mesilate were administrated along with nutritional support. The pancreatic and duodenal fistula had been producing duodenal juice for over 30 days since the re-operative surgery. As suspected, reduced FXIII activity was confirmed in the patient. After administering FXIII for 5 days, the amount of duodenal juice from the fistula markedly reduced, and the fistula closed immediately afterwards. The results of our study suggest that administration of FXIII could be a reasonable and effective treatment for patients with pancreatic or/and enterocutaneous fistula who are resistant to standard treatments.

Metastatic adenocarcinoma of mammary-like glands of the vulva successfully treated with surgery and hormonal therapy.

PubMed

Benito, Virginia; Arribas, Sara; Martínez, David; Medina, Norberto; Lubrano, Amina; Arencibia, Octavio

2013-01-01

Vulvar cancer is a rare malignancy; most tumors are squamous cell type while adenocarcinomas are rare. Primary adenocarcinomas of the vulva predominantly include extramammary Paget's disease and sweat gland carcinomas. Greene first described a rare form of adenocarcinoma in 1936, which was called adenocarcinoma of mammary-like glands of the vulva because of its morphologic and immunohistochemical resemblance to breast adenocarcinomas. In the management of this entity, varying combinations of surgery, radiation therapy, systemic chemotherapy and/or hormonal therapy may be used, as in patients with orthotopic breast carcinoma. However, hormonal therapy leads the way in patients with positive hormonal receptors, where other therapies cannot be used due to comorbidities or advanced age. We present the first reported case of an elderly patient with metastatic vulvar adenocarcinoma arising from mammary-like glands, successfully treated with a combination of surgery and hormonal therapy. © 2012 The Authors. Journal of Obstetrics and Gynaecology Research © 2012 Japan Society of Obstetrics and Gynecology.

Intractable depression successfully treated with a combination of autogenic training and high-dose antidepressant in department of otorhinolaryngology: a case report.

PubMed

Goto, Fumiyuki; Nakai, Kimiko; Murakami, Masato; Ogawa, Kaoru

2009-08-14

Patients suffering from ear discomfort are commonly encountered in the department of otolaryngology. If various clinical examinations do not reveal any objective findings, then the patients are referred to the department of internal medicine or psychiatry. Psychotherapy is recommended in some cases. This paper describes the successful administration of autogenic training in a patient suffering from ear discomfort due to major depression. We present a case of intractable depression that was successfully treated with a combination of psychotherapy, administered by a clinical psychologist, and high-dose antidepressant. The patient was a 36-year-old female with hearing discomfort in her left ear. In 2003, she experienced insomnia and an appetite loss, and her condition was diagnosed as major depression along with an avoidant personality disorder. Her depression has not been improved with antidepressant treatment for 3 years in department of psychosomatic medicine. She was referred to our department because of ear discomfort in her left ear. There was no abnormality in her physical examinations. She wanted to be treated in department of otorhinolaryngology. We increased the dose of fluvoxamine maleate up to 200 mg/day, and introduced cognitive therapy and autogenic training by a clinical psychologist. Eventually, her depressive state as well as the hearing complaint was markedly alleviated. Autogenic training can be a viable and acceptable treatment option for patients who fail to respond to other therapies. This case emphasizes the importance of autogenic training as a method to control physical symptom of depression.

Non-Thyroidal Illness Syndrome in Patients Exposed to Indoor Air Dampness Microbiota Treated Successfully with Triiodothyronine

PubMed Central

Somppi, Taija Liisa

2017-01-01

Long-term exposure to dampness microbiota induces multi-organ morbidity. One of the symptoms related to this disorder is non-thyroidal illness syndrome (NTIS). A retrospective study was carried out in nine patients with a history of mold exposure, experiencing chronic fatigue, cognitive disorder, and different kinds of hypothyroid symptoms despite provision of levothyroxine (3,5,3′,5′-tetraiodothyronine, LT4) monotherapy. Exposure to volatile organic compounds present in water-damaged buildings including metabolic products of toxigenic fungi and mold-derived inflammatory agents can lead to a deficiency or imbalance of many hormones, such as active T3 hormone. Since the 1970s, the synthetic prohormone, levothyroxine (LT4), has been the most commonly prescribed thyroid hormone in replacement monotherapy. It has been presumed that the peripheral conversion of T4 (3,5,3′,5′-tetraiodothyronine) into T3 (3,5,3′-triiodothyronine) is sufficient to satisfy the overall tissue requirements. However, evidence is presented that this not the case for all patients, especially those exposed to indoor air molds. This retrospective study describes the successful treatment of nine patients in whom NTIS was treated with T3-based thyroid hormone. The treatment was based on careful interview, clinical monitoring, and laboratory analysis of serum free T3 (FT3), reverse T3 (rT3) and thyroid-stimulating hormone, free T4, cortisol, and dehydroepiandrosterone (DHEA) values. The ratio of FT3/rT3 was calculated. In addition, some patients received adrenal support with hydrocortisone and DHEA. All patients received nutritional supplementation and dietary instructions. During the therapy, all nine patients reported improvements in all of the symptom groups. Those who had residual symptoms during T3-based therapy remained exposed to indoor air molds in their work places. Four patients were unable to work and had been on disability leave for a long time during LT4 monotherapy. However

Surgically Induced Necrotizing Scleritis Following Strabismus Surgery Treated Successfully with Topical N-acetylcysteine in a Child with Congenital Fibrosis of Extraocular Muscles and Varadi Papp Syndrome.

PubMed

Rajamani, Muralidhar; Nagasubramanian, Vidhya; Ayyavoo, Ahila; Raghupathy, Palany; Dandapani, Ramamurthy

2017-03-01

Surgically induced necrotizing scleritis (SINS) is a rare but serious disorder that can develop many years after strabismus surgery. It is generally treated with high-dose steroids or immunosuppression. We describe a patient with Varadi Papp syndrome and congenital fibrosis of the extraocular muscles, who developed surgically induced necrotizing scleritis a month after strabismus surgery and was successfully managed by oral vitamin C and topical N-acetylcysteine 10%. While SINS is conventionally treated with steroids/immunosuppression, a conservative approach may be tried in milder cases. The role of topical N-acetylcysteine in managing this complication needs to be explored.

MPLW515L Is a Novel Somatic Activating Mutation in Myelofibrosis with Myeloid Metaplasia

PubMed Central

Pikman, Yana; Lee, Benjamin H; Mercher, Thomas; McDowell, Elizabeth; Ebert, Benjamin L; Gozo, Maricel; Cuker, Adam; Wernig, Gerlinde; Moore, Sandra; Galinsky, Ilene; DeAngelo, Daniel J; Clark, Jennifer J; Lee, Stephanie J; Golub, Todd R; Wadleigh, Martha; Gilliland, D. Gary; Levine, Ross L

2006-01-01

Background The JAK2V617F allele has recently been identified in patients with polycythemia vera (PV), essential thrombocytosis (ET), and myelofibrosis with myeloid metaplasia (MF). Subsequent analysis has shown that constitutive activation of the JAK-STAT signal transduction pathway is an important pathogenetic event in these patients, and that enzymatic inhibition of JAK2V617F may be of therapeutic benefit in this context. However, a significant proportion of patients with ET or MF are JAK2V617F-negative. We hypothesized that activation of the JAK-STAT pathway might also occur as a consequence of activating mutations in certain hematopoietic-specific cytokine receptors, including the erythropoietin receptor (EPOR), the thrombopoietin receptor (MPL), or the granulocyte-colony stimulating factor receptor (GCSFR). Methods and Findings DNA sequence analysis of the exons encoding the transmembrane and juxtamembrane domains of EPOR, MPL, and GCSFR, and comparison with germline DNA derived from buccal swabs, identified a somatic activating mutation in the transmembrane domain of MPL (W515L) in 9% (4/45) of JAKV617F-negative MF. Expression of MPLW515L in 32D, UT7, or Ba/F3 cells conferred cytokine-independent growth and thrombopoietin hypersensitivity, and resulted in constitutive phosphorylation of JAK2, STAT3, STAT5, AKT, and ERK. Furthermore, a small molecule JAK kinase inhibitor inhibited MPLW515L-mediated proliferation and JAK-STAT signaling in vitro. In a murine bone marrow transplant assay, expression of MPLW515L, but not wild-type MPL, resulted in a fully penetrant myeloproliferative disorder characterized by marked thrombocytosis (Plt count 1.9–4.0 × 10 12/L), marked splenomegaly due to extramedullary hematopoiesis, and increased reticulin fibrosis. Conclusions Activation of JAK-STAT signaling via MPLW515L is an important pathogenetic event in patients with JAK2V617F-negative MF. The bone marrow transplant model of MPLW515L-mediated myeloproliferative

MPLW515L is a novel somatic activating mutation in myelofibrosis with myeloid metaplasia.

PubMed

Pikman, Yana; Lee, Benjamin H; Mercher, Thomas; McDowell, Elizabeth; Ebert, Benjamin L; Gozo, Maricel; Cuker, Adam; Wernig, Gerlinde; Moore, Sandra; Galinsky, Ilene; DeAngelo, Daniel J; Clark, Jennifer J; Lee, Stephanie J; Golub, Todd R; Wadleigh, Martha; Gilliland, D Gary; Levine, Ross L

2006-07-01

The JAK2V617F allele has recently been identified in patients with polycythemia vera (PV), essential thrombocytosis (ET), and myelofibrosis with myeloid metaplasia (MF). Subsequent analysis has shown that constitutive activation of the JAK-STAT signal transduction pathway is an important pathogenetic event in these patients, and that enzymatic inhibition of JAK2V617F may be of therapeutic benefit in this context. However, a significant proportion of patients with ET or MF are JAK2V617F-negative. We hypothesized that activation of the JAK-STAT pathway might also occur as a consequence of activating mutations in certain hematopoietic-specific cytokine receptors, including the erythropoietin receptor (EPOR), the thrombopoietin receptor (MPL), or the granulocyte-colony stimulating factor receptor (GCSFR). DNA sequence analysis of the exons encoding the transmembrane and juxtamembrane domains of EPOR, MPL, and GCSFR, and comparison with germline DNA derived from buccal swabs, identified a somatic activating mutation in the transmembrane domain of MPL (W515L) in 9% (4/45) of JAKV617F-negative MF. Expression of MPLW515L in 32D, UT7, or Ba/F3 cells conferred cytokine-independent growth and thrombopoietin hypersensitivity, and resulted in constitutive phosphorylation of JAK2, STAT3, STAT5, AKT, and ERK. Furthermore, a small molecule JAK kinase inhibitor inhibited MPLW515L-mediated proliferation and JAK-STAT signaling in vitro. In a murine bone marrow transplant assay, expression of MPLW515L, but not wild-type MPL, resulted in a fully penetrant myeloproliferative disorder characterized by marked thrombocytosis (Plt count 1.9-4.0 x 10(12)/L), marked splenomegaly due to extramedullary hematopoiesis, and increased reticulin fibrosis. Activation of JAK-STAT signaling via MPLW515L is an important pathogenetic event in patients with JAK2V617F-negative MF. The bone marrow transplant model of MPLW515L-mediated myeloproliferative disorders (MPD) exhibits certain features of human MF

[Two cases of afferent loop syndrome caused by obstruction at the jejuno-jejunostomy site in the Roux-en-Y loop that were successfully treated by endoscopic balloon dilatation].

PubMed

Yasuda, Atsushi; Imamoto, Haruhiko; Furukawa, Hiroshi; Imano, Motohiro; Yasuda, Takushi; Okuno, Kiyokata

2014-11-01

We report 2 rare cases of afferent loop syndrome caused by obstruction at the jejuno-jejunostomy site in the Roux-en-Y loop after total gastrectomy, which was successfully treated by endoscopic balloon dilatation of the anastomotic stenosis. Case 1: A 62-year-old woman presented with malaise and lower abdominal distension 6 months after laparoscopy-assisted total gastrectomy with Roux-en-Y reconstruction. She was diagnosed with afferent loop syndrome; CT imaging indicated marked dilatation of the afferent loop, with membranous obstruction at the jejuno-jejunostomy site in the Roux-en-Y loop. Although almost complete occlusion was noted at the jejuno-jejunostomy site, the obstruction was successfully relieved by endoscopic balloon dilation using TandemTM XL Triple Lumen ERCP Cannula (Boston Scientific)®. Case 2: A 70-year-old man presented with malaise and lower abdominal distension 3 years after laparoscopy-assisted total gastrectomy with Roux-en-Y reconstruction. He was diagnosed with afferent loop syndrome; CT imaging indicated complete obstruction at the jejuno-jejunostomy site in the Roux-en-Y loop. As in case 1, the obstruction was successfully treated by endoscopic balloon dilatation of the occluded anastomosis.

Craniospinal Germinomas in Patient with Down Syndrome Successfully Treated with Standard-Dose Chemotherapy and Craniospinal Irradiation: Case Report and Literature Review.

PubMed

Miyake, Yohei; Adachi, Jun-Ichi; Suzuki, Tomonari; Mishima, Kazuhiko; Sasaki, Atsushi; Nishikawa, Ryo

2017-12-01

Patients with Down syndrome (DS) are more likely to develop chemotherapy-related complications. The standard treatment for these patients with cancer has not yet been established, and the risks of standard chemotherapy are unclear. In this paper, a rare case of multiple craniospinal germinomas in a patient with DS, which was successfully treated with standard-dose chemotherapy combined with craniospinal irradiation, is reported. The authors report a case of multiple craniospinal germinomas in a DS patient who presented with bilateral oculomotor and facial nerve palsy and hearing loss. The patient underwent 3 courses of combination chemotherapy using a standard dose of carboplatin and etoposide and 23.4 Gy of concurrent craniospinal irradiation. Posttreatment magnetic resonance imaging showed reduction of the tumors. Both fluorodeoxyglucose- and methionine-positron emission tomography demonstrated no uptake in the residual tumors. Follow-up magnetic resonance imaging and positron emission tomography did not reveal tumor recurrence for 18 months. As far as we know, this is the first case of multiple craniospinal germinomas in a patient with DS who achieved a successful treatment result without fatal adverse events. The literature review indicated that disseminated germinomas may need intensive treatment to reduce recurrence risk. However, intensive chemotherapy using a combination of 3 or more anticancer drugs can increase the rate of treatment-related death during the early stage. Our case indicated that multiple craniospinal germinoma of DS patients could be treated with a standard dose of carboplatin and etoposide regimen with concurrent craniospinal irradiation along with appropriate supportive therapy and careful observation. Copyright © 2017 Elsevier Inc. All rights reserved.

Intractable depression successfully treated with a combination of autogenic training and high-dose antidepressant in department of otorhinolaryngology: a case report

PubMed Central

2009-01-01

Introduction Patients suffering from ear discomfort are commonly encountered in the department of otolaryngology. If various clinical examinations do not reveal any objective findings, then the patients are referred to the department of internal medicine or psychiatry. Psychotherapy is recommended in some cases. This paper describes the successful administration of autogenic training in a patient suffering from ear discomfort due to major depression. Case presentation We present a case of intractable depression that was successfully treated with a combination of psychotherapy, administered by a clinical psychologist, and high-dose antidepressant. The patient was a 36-year-old female with hearing discomfort in her left ear. In 2003, she experienced insomnia and an appetite loss, and her condition was diagnosed as major depression along with an avoidant personality disorder. Her depression has not been improved with antidepressant treatment for 3 years in department of psychosomatic medicine. She was referred to our department because of ear discomfort in her left ear. There was no abnormality in her physical examinations. She wanted to be treated in department of otorhinolaryngology. We increased the dose of fluvoxamine maleate up to 200 mg/day, and introduced cognitive therapy and autogenic training by a clinical psychologist. Eventually, her depressive state as well as the hearing complaint was markedly alleviated. Conclusion Autogenic training can be a viable and acceptable treatment option for patients who fail to respond to other therapies. This case emphasizes the importance of autogenic training as a method to control physical symptom of depression. PMID:20184684

Vorinostat, Cytarabine, and Etoposide in Treating Patients With Relapsed and/or Refractory Acute Leukemia or Myelodysplastic Syndromes or Myeloproliferative Disorders

ClinicalTrials.gov

2013-05-01

Accelerated Phase Chronic Myelogenous Leukemia; Adult Acute Basophilic Leukemia; Adult Acute Eosinophilic Leukemia; Adult Acute Megakaryoblastic Leukemia (M7); Adult Acute Minimally Differentiated Myeloid Leukemia (M0); Adult Acute Monoblastic Leukemia (M5a); Adult Acute Monocytic Leukemia (M5b); Adult Acute Myeloblastic Leukemia With Maturation (M2); Adult Acute Myeloblastic Leukemia Without Maturation (M1); Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Adult Acute Myelomonocytic Leukemia (M4); Adult Acute Promyelocytic Leukemia (M3); Adult Erythroleukemia (M6a); Adult Pure Erythroid Leukemia (M6b); Atypical Chronic Myeloid Leukemia, BCR-ABL1 Negative; Blastic Phase Chronic Myelogenous Leukemia; Chronic Eosinophilic Leukemia; Chronic Myelomonocytic Leukemia; Chronic Neutrophilic Leukemia; de Novo Myelodysplastic Syndromes; Essential Thrombocythemia; Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable; Polycythemia Vera; Previously Treated Myelodysplastic Syndromes; Primary Myelofibrosis; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Relapsing Chronic Myelogenous Leukemia; Secondary Acute Myeloid Leukemia; Secondary Myelodysplastic Syndromes

Acquired 5-oxoproline acidemia successfully treated with N-acetylcysteine.

PubMed

Hundemer, Gregory L; Fenves, Andrew Z

2017-04-01

Acquired 5-oxoprolinemia is increasingly recognized as a cause of anion gap metabolic acidosis. It predominantly occurs in chronically ill, malnourished women with impaired renal function and chronic acetaminophen ingestion. Depletion of glutathione and cysteine stores leads to elevated 5-oxoproline levels. N-acetylcysteine, given its effect in repleting glutathione and cysteine stores, has been proposed as a potential treatment for 5-oxoprolinemia, though reports of its successful use are lacking. We present a case of 5-oxoproline metabolic acidosis that persisted despite discontinuation of acetaminophen. However, the acidosis rapidly resolved with N-acetylcysteine administration.

A Phase 1/2 Study To Evaluate ASN002 In Relapsed/Refractory Lymphoma And Advanced Solid Tumors

ClinicalTrials.gov

2018-04-30

Lymphoma, Large B-Cell, Diffuse; Lymphoma, Mantle-Cell; Lymphoma, Follicular; Cancer; Neoplasm; Tumor; Lymphoma, Malignant; Lymphoma, B-cell; Lymphoma, Non-Hodgkin; B-Cell Chronic Lymphocytic Leukemia; B-Cell Leukemia, Chronic; B-Lymphocytic Leukemia, Chronic; Chronic Lymphocytic Leukemia; Leukemia, Lymphocytic, Chronic; Leukemia, Lymphocytic, Chronic, B Cell; Myelofibrosis; Chronic Idiopathic Myelofibrosis; Idiopathic Myelofibrosis; Lymphoma, T Cell, Peripheral; Peripheral T-Cell Lymphoma; T-Cell Lymphoma, Peripheral

Hemolytic Uremic Syndrome-associated Encephalopathy Successfully Treated with Corticosteroids.

PubMed

Hosaka, Takashi; Nakamagoe, Kiyotaka; Tamaoka, Akira

2017-11-01

The encephalopathy that occurs in association with hemolytic uremic syndrome (HUS), which is caused by enterohemorrhagic Escherichia coli (E. coli), has a high mortality rate and patients sometimes present sequelae. We herein describe the case of a 20-year-old woman who developed encephalopathy during the convalescent stage of HUS caused by E.coli O26. Hyperintense lesions were detected in the pons, basal ganglia, and cortex on diffusion-weighted brain MRI. From the onset of HUS encephalopathy, we treated the patient with methylprednisolone (mPSL) pulse therapy alone. Her condition improved, and she did not present sequelae. Our study shows that corticosteroids appear to be effective for the treatment of some patients with HUS encephalopathy.

EGFR-mutant Non-small Cell Lung Cancer Accompanied by Transient Asymptomatic Pulmonary Opacities Successfully Treated with "Stop-And-Go" Osimertinib.

PubMed

Kobayashi, Keigo; Naoki, Katsuhiko; Kuroda, Aoi; Yasuda, Hiroyuki; Kawada, Ichiro; Soejima, Kenzo; Betsuyaku, Tomoko

2018-04-01

A 69-year-old man with post-operative recurrence of lung adenocarcinoma was treated with multiple chemotherapies, including epidermal growth factor receptor (EGFR)-tyrosine kinase inhibitors. A second biopsy revealed an EGFR T790M mutation. As 10th-line chemotherapy, osimertinib was initiated. After 24 weeks, chest computed tomography (CT) revealed asymptomatic ground-glass opacities in both lobes. After four weeks of osimertinib discontinuation, imaging revealed rapid lung cancer progression. Osimertinib was resumed. After 11 weeks, CT revealed decreased lung nodules with no exacerbation of interstitial lung disease. We describe a patient who experienced transient asymptomatic pulmonary opacities during treatment with osimertinib, which was successfully managed by a "stop-and-go" approach.

Adult cystic hygroma: successful use of OK-432 (Picibanil).

PubMed

Woolley, S L; Smith, D R K; Quine, S

2008-11-01

We report an adult case of cystic lymphangioma treated with OK-432 (Picibanil). A case report and review of the literature concerning the use of OK-432 to treat cystic lymphangioma is presented. A 31-year-old woman developed a cystic lymphangioma four weeks post-partum. This was treated initially by aspiration, for diagnostic purposes. Investigation suggested that surgery would be challenging. A review of the literature demonstrated success with OK-432 in the treatment of this condition, although primarily in the paediatric population. This patient was successfully treated thus, and at the time of writing remained symptom free. A suggested management plan is outlined. Treatment with OK-432 is useful in the management of cystic lymphangiomas in adults and should be considered as first line treatment.

HIV seronegative eosinophilic pustular folliculitis successfully treated with doxicycline.

PubMed

Brazzelli, V; Barbagallo, T; Prestinari, F; Ciocca, O; Vassallo, C; Borroni, G

2004-07-01

Eosinophilic pustular folliculitis (EPF) is an unusual disease, first described in adult East Asians in 1970 by Ofuji. It is characterized by follicular papules and pustules tending to coalesce and form plaques involving the trunk, face and extremities. In recent years, it has been often associated with human immunodeficiency virus (HIV) infection or with immunosuppressed and/or oncohaematological patients. EPF has been described in immunocompetent adult caucasian patients only occasionally. The diagnosis requires clinical and microbiological features such as sterile folliculitis and histopathological findings characterized by folliculitis and perifolliculitis with eosinophilic infiltrate. We describe an HIV seronegative caucasian male with EPF, allergic to non-steroidal anti-inflammatory drugs and indomethacin, treated with oral doxicycline. The treatment led to the complete remission of the lesions within 2 months.

[Tentorial Dural Arteriovenous Fistula Successfully Treated with Transvenous Embolization Using a Double Catheterization Technique through Venous Drainage:A Case Report].

PubMed

Yamakawa, Akira; Fujita, Atsushi; Tanaka, Hirotomo; Ikeda, Mitsuru; Morikawa, Masashi; Kohmura, Eiji

2017-07-01

Tentorial dural arteriovenous fistulas(dAVFs)are a rare clinical entity accounting for less than 10% of all intracranial dAVFs. Because these lesions are characterized by high hemorrhagic risk, aggressive treatment should be considered. Although the number of reported cases treated with endovascular transarterial embolization(TAE)using glue has been increasing, little is known about the transvenous approach. Here, we report the case of a patient with a tentorial dAVF who was successfully treated with transvenous embolization(TVE)through venous drainage using a double catheterization technique. A 68-year-old male patient who had a history of left putaminal hemorrhage treated with a craniotomy was diagnosed with a tentorial dAVF on a magnetic resonance angiogram. Because the patient refused another craniotomy for surgical interruption of the dAVF, an endovascular approach was considered. We first attempted to perform TAE with glue, but catheterization into the tortuous meningohypophyseal trunk failed. We then performed a TVE of the venous drainage near the shunt with detachable coils and achieved complete obliteration of the fistula. During coil embolization of the venous drainage, insertion of small coils near the shunt was supported by another anchor coil that was delivered using a double catheterization technique. The method of TVE through venous drainage using a double catheterization technique, which involved placing coils in the fragile drainage vein, was safe and effective in a case of tentorial dAVF. This technique should be considered as another option for the management of complex tentorial dAVFs.

Successful treatment of poison oak dermatitis treated with Grindelia spp. (Gumweed).

PubMed

Canavan, Don; Yarnell, Eric

2005-08-01

Poison oak and related hypersensitivity dermatitides are age-old problems that have historically been treated with herbal medicines before the availability of corticosteroids. Few of these historical therapies have been rigorously investigated. The case presented here provides some insight into the potential efficacy of certain herbs for relieving mild-to-moderate poison ivy dermatitis.

Sarcoptic mange in three alpacas treated successfully with amitraz.

PubMed

Lau, Peri; Hill, Peter B; Rybnícek, Jan; Steel, Lynne

2007-08-01

Sarcoptic mange is a serious skin disease in alpacas that can result in high morbidity and even mortality. Three alpacas were presented with sarcoptic mange that had previously failed to respond to repeated topical applications of eprinomectin, and an injection of doramectin. They were moderately to severely pruritic, had extensive lesions of alopecia, erythema, scaling and crusting, and had lost weight. As no drug is currently licensed for the treatment of sarcoptic mange in alpacas in the UK, they were treated with a topical solution of amitraz (50 mL in 10 L) after initial bathing with antibacterial or keratolytic shampoos. The clinical signs completely resolved with no relapse over a 10-month follow-up period. In this small group of alpacas, amitraz was an effective and well-tolerated treatment for sarcoptic mange.

Extensive Nevus Comedonicus, Complicated with Recurrent Abscesses, Successfully Treated with Surgical Resurfacing.

PubMed

Manikavachakan, Narender; Siddaraju, Maheshwari Nallur; Rajendran, Sarangapani Chakrapani; Venkataraman, Aniketh

2018-01-01

Nevus comedonicus is a rare epidermal abnormality of the pilosebaceous unit, which is congenital in most patients but may also appear early in childhood. It may be localized or have an extensive involvement, the latter showing a unilateral predominance with only a few cases presenting bilaterally. Extensive nevus comedonicus can be associated with musculoskeletal defects, eye and neurological involvement, which constitutes nevus comedonicus syndrome. Uncomplicated nevus comedonicus can be treated with topical keratolytics, diode, erbium laser, and ultrapulse CO 2 laser. Surgical excision can be performed to ensure complete removal and nonrecurrence. This case report refers to a young male patient with extensive nevus comedonicus present over left chest, left axilla, and left upper back without systemic involvement, treated with staged surgical excision and resurfacing.

Primary diffuse large B cell lymphoma of the uterine cervix successfully treated by combined chemotherapy alone

PubMed Central

Cubo, Ana María; Soto, Zandra Mileny; Cruz, Miguel Ángel; Doyague, María José; Sancho, Verónica; Fraino, Aurymar; Blanco, Óscar; Puig, Noemi; Alcoceba, Miguel; González, Marcos; Sayagués, José María

2017-01-01

Abstract Rationale: Primary lymphomas of the uterine cervix are a rare disease. They are often misdiagnosed because of their rarity and because they can be easily confused with a squamous cell carcinoma of the cervix, as they are usually presented as exophytic mass with vaginal bleeding as their most common symptoms. Nevertheless, considering that both the prognosis and the treatment are completely different between them, differential diagnosis should be taken into account. Patient concerns: A case of a 51-year-old woman with a primary diffuse large B-cell lymphoma of the cervix is presented. Diagnoses: Diagnosis of this tumor was a challenge for pathologists and clinicians, as four biopsies were needed to achieve a final diagnosis. Interventions: Patient was successfully treated with combined Rituximab and chemotherapy (R-CHOP) alone. Outcomes: Complete remission, confirmed through biopsy, was reached after six courses of chemotherapy. At 2-years follow up, patient is alive and free of disease. Lessons: Considering that the prognosis and treatment of primary malignant lymphoma of the cervix are completely different than that of the squamous cell carcinoma, awareness of this disease should be considered in the differential diagnosis. PMID:28489772

Aplastic anemia, mucormycosis and aspergillosis in infectious mononucleosis: success is possible.

PubMed

Ergene, Ulku; Ozbalci, Demircan; Baykal, Bahattin; Metin Ciris, I; Yariktas, Murat

2007-10-01

Infectious mononucleosis (IM) is a rare cause of aplastic anemia in adults. We report of a patient in whom aplastic anemia, mucormycosis and aspergillosis complicated during the course of IM and successfully treated with liposomal amphotericin B. According to our searches in literature, we could not find a similar patient complicated and successfully treated like ours.

Philadelphia chromosome-negative myeloproliferative disorders: biology and treatment.

PubMed

Hoffman, Ronald; Prchal, Josef T; Samuelson, Scott; Ciurea, Stefan O; Rondelli, Damiano

2007-01-01

The Philadelphia chromosome (Ph)-negative myeloproliferative disorders (MPDs) include essential thrombocythemia (ET), idiopathic myelofibrosis (IMF), and polycythemia vera (PV). All of these disorders are clonal hematologic malignancies originating at the level of the pluripotent hematopoietic stem cell. Recently, activating mutations of the intracellular cytokine-signaling molecule JAK2 have been identified in > 90% of patients with PV and in 50% of those with IMF and ET. In addition, a mutation of the thrombopoietin receptor, MPLW515L, has been documented in some patients with IMF. Both mutations activate JAK-STAT signaling pathways and likely play a role in disease progression. Both ET and PV are associated with prolonged clinical courses associated with frequent thrombotic and hemorrhagic events, and progression to myelofibrosis and acute leukemia. IMF has a much poorer prognosis and is associated with cytopenias, splenomegaly, extramedullary hematopoiesis, and bone marrow fibrosis. Stratification of risk for the development of complications from Ph-negative MPDs has guided the identification of appropriate therapies for this population. Intermediate/high-risk IMF or myelofibrosis after ET or PV is associated with a sufficiently poor prognosis to justify the use of allogeneic stem cell transplantation, which is capable of curing such patients. Reduced-intensity conditioning in preparation for allogeneic stem cell transplantation has permitted older patients with IMF to undergo transplantation with increasing success.

EGFR-mutant Non-small Cell Lung Cancer Accompanied by Transient Asymptomatic Pulmonary Opacities Successfully Treated with “Stop-And-Go” Osimertinib

PubMed Central

Kobayashi, Keigo; Naoki, Katsuhiko; Kuroda, Aoi; Yasuda, Hiroyuki; Kawada, Ichiro; Soejima, Kenzo; Betsuyaku, Tomoko

2017-01-01

A 69-year-old man with post-operative recurrence of lung adenocarcinoma was treated with multiple chemotherapies, including epidermal growth factor receptor (EGFR)-tyrosine kinase inhibitors. A second biopsy revealed an EGFR T790M mutation. As 10th-line chemotherapy, osimertinib was initiated. After 24 weeks, chest computed tomography (CT) revealed asymptomatic ground-glass opacities in both lobes. After four weeks of osimertinib discontinuation, imaging revealed rapid lung cancer progression. Osimertinib was resumed. After 11 weeks, CT revealed decreased lung nodules with no exacerbation of interstitial lung disease. We describe a patient who experienced transient asymptomatic pulmonary opacities during treatment with osimertinib, which was successfully managed by a “stop-and-go” approach. PMID:29269665

Pseudocyst in the pancreatic tail associated with chronic pancreatitis successfully treated by transpapillary cyst drainage.

PubMed

Naitoh, Itaru; Ohara, Hirotaka; Okayama, Yasutaka; Nakazawa, Takahiro; Ando, Tomoaki; Hayashi, Kazuki; Okumura, Fumihiro; Kitajima, Yasuhiro; Ban, Tessin; Miyabe, Katsuyuki; Ueno, Koichiro; Joh, Takashi; Sano, Hitoshi

2008-09-01

We report a 50-year-old male with pseudocysts in the pancreatic tail associated with chronic pancreatitis successfully treated by transpapillary cyst drainage. He had previously undergone ultrasonography-guided percutaneous cyst drainage for a pancreatic pseudocyst in our hospital. He was readmitted due to abdominal pain and fever. Computed tomography showed recurrence of a pseudocyst in the pancreatic tail measuring 5 cm in diameter. Since conservative treatment failed, endoscopic retrograde pancreatography was performed. There was communication between the pseudocyst and the main pancreatic duct, and pancreatic duct stenosis proximal to the pseudocyst. First, transpapillary pancreatic duct drainage was performed using a plastic stent, but the pseudocyst did not decrease in size and became infected. After removal of the stent, a pigtail type nasocystic catheter was placed in the pseudocyst via the pancreatic duct. The pseudocyst infection immediately disappeared, and the pseudocyst gradually decreased and disappeared. After removal of the nasocystic catheter, no recurrence was observed. As transpapillary drainage of pancreatic pseudocyst, cyst drainage and pancreatic duct drainage have been reported. In our patient with pseudocyst in the pancreatic tail, duct drainage was ineffective and the pseudocyst was infected, whereas cyst drainage was very effective. We considered that cyst drainage by a nasocystic catheter was the first-line therapy as the transpapillary drainage of the pancreatic pseudocyst.

Pseudocyst in the Pancreatic Tail Associated with Chronic Pancreatitis Successfully Treated by Transpapillary Cyst Drainage

PubMed Central

Naitoh, Itaru; Ohara, Hirotaka; Okayama, Yasutaka; Nakazawa, Takahiro; Ando, Tomoaki; Hayashi, Kazuki; Okumura, Fumihiro; Kitajima, Yasuhiro; Ban, Tessin; Miyabe, Katsuyuki; Ueno, Koichiro; Joh, Takashi; Sano, Hitoshi

2008-01-01

We report a 50-year-old male with pseudocysts in the pancreatic tail associated with chronic pancreatitis successfully treated by transpapillary cyst drainage. He had previously undergone ultrasonography-guided percutaneous cyst drainage for a pancreatic pseudocyst in our hospital. He was readmitted due to abdominal pain and fever. Computed tomography showed recurrence of a pseudocyst in the pancreatic tail measuring 5 cm in diameter. Since conservative treatment failed, endoscopic retrograde pancreatography was performed. There was communication between the pseudocyst and the main pancreatic duct, and pancreatic duct stenosis proximal to the pseudocyst. First, transpapillary pancreatic duct drainage was performed using a plastic stent, but the pseudocyst did not decrease in size and became infected. After removal of the stent, a pigtail type nasocystic catheter was placed in the pseudocyst via the pancreatic duct. The pseudocyst infection immediately disappeared, and the pseudocyst gradually decreased and disappeared. After removal of the nasocystic catheter, no recurrence was observed. As transpapillary drainage of pancreatic pseudocyst, cyst drainage and pancreatic duct drainage have been reported. In our patient with pseudocyst in the pancreatic tail, duct drainage was ineffective and the pseudocyst was infected, whereas cyst drainage was very effective. We considered that cyst drainage by a nasocystic catheter was the first-line therapy as the transpapillary drainage of the pancreatic pseudocyst. PMID:21897788

Failure after Success: Correlates of Recidivism among Individuals Who Successfully Completed Coerced Drug Treatment

ERIC Educational Resources Information Center

Sung, Hung-En; Belenko, Steven

2005-01-01

A subset of criminal offenders diverted from prison to treatment return to crime after successful completion of treatment. Identifying correlates of recidivism among treatment completers will improve our ability to help treated offenders to better capitalize on their treatment experiences. Data from 156 mandated clients of long-term residential…

Myelofibrosis

MedlinePlus

... into all of your blood cells. Your blood is made of: Red blood cells (which carry oxygen to your tissues) White blood cells (which fight infection) Platelets (which help your blood clot) When the bone marrow is scarred, it cannot make enough blood cells. Anemia , ...

Factors predicting the duration of adrenal insufficiency in patients successfully treated for Cushing disease and nonmalignant primary adrenal Cushing syndrome.

PubMed

Prete, Alessandro; Paragliola, Rosa Maria; Bottiglieri, Filomena; Rota, Carlo Antonio; Pontecorvi, Alfredo; Salvatori, Roberto; Corsello, Salvatore Maria

2017-03-01

Successful treatment of Cushing syndrome causes transient or permanent adrenal insufficiency deriving from endogenous hypercortisolism-induced hypothalamus-pituitary-adrenal-axis suppression. We analyzed pre-treatment factors potentially affecting the duration of adrenal insufficiency. We conducted a retrospective analysis on patients successfully treated for Cushing disease (15 patients) who underwent transsphenoidal surgery, and nonmalignant primary adrenal Cushing syndrome (31 patients) who underwent unilateral adrenalectomy, divided into patients with overt primary adrenal Cushing syndrome (14 patients) and subclinical primary adrenal Cushing syndrome (17 patients). Epidemiological data, medical history, and hormonal parameters depending on the etiology of hypercortisolism were collected and compared to the duration of adrenal insufficiency. The median duration of follow-up after surgery for Cushing disease and primary adrenal Cushing syndrome was 70 and 48 months, respectively. In the Cushing disease group, the median duration of adrenal insufficiency after transsphenoidal surgery was 15 months: younger age at diagnosis and longer duration of signs and symptoms of hypercortisolism before diagnosis and surgery were associated with longer duration of adrenal insufficiency. The median duration of adrenal insufficiency was 6 months for subclinical primary adrenal Cushing syndrome and 18.5 months for overt primary adrenal Cushing syndrome. The biochemical severity of hypercortisolism, the grade of hypothalamus-pituitary-adrenal-axis suppression, and treatment with ketoconazole before surgery accounted for longer duration of adrenal insufficiency. In patients with Cushing disease, younger age and delayed diagnosis and treatment predict longer need for glucocorticoid replacement therapy after successful transsphenoidal surgery. In patients with primary adrenal Cushing syndrome, the severity of hypercortisolism plays a primary role in influencing the duration of

23 Lung Metastases Treated by Radiofrequency Ablation Over 10 Years in a Single Patient: Successful Oncological Outcome of a Metastatic Cancer Without Altered Respiratory Function

DOE Office of Scientific and Technical Information (OSTI.GOV)

Crombé, Amandine, E-mail: amandine.crombe@ens-lyon.fr; Buy, Xavier; Godbert, Yann

An 82-year-old man, who was diagnosed in 2002 with an oncocytic (Hürthle cell) thyroid carcinoma, was initially treated by local surgery and was refractory to radioiodine treatment. The patient had successive secondary recurrences from 2006 onwards. Metastases were suspected due to an elevation of thyroglobulin in serum. Hypermetabolic nodules were targeted using FDG PET as well as CT-guided radiofrequency ablations. Thyroglobulin levels decreased following each procedure. 10 years later, tolerance and efficacy are excellent; 23 lung metastases have been treated during 11 sessions without current relapse. Respiratory function and quality of life are not altered. This report illustrates how radiofrequencymore » ablation can be efficiently integrated into the long-term management of poorly aggressive oligometastatic cancer, in combination with other local and/or systemic therapies.« less

Giant Hepatic Aneurysm Presenting With Hematemesis Successfully Treated With an Endovascular Technique.

PubMed

Abdallah, Feras F; Serracino-Inglott, Ferdinand; Ananthakrishnan, Ganapathy

2017-07-01

Hepatic artery aneurysms are uncommon visceral aneurysms that are usually found incidentally on imaging. We present a case of large common hepatic aneurysm presenting with life-threatening hematemesis due to duodenal erosion, in a 66-year-old man, treated by embolization with Onyx and coils while preserving hepatic perfusion.

The role of JAK1/2 inhibitors in the treatment of chronic myeloproliferative neoplasms.

PubMed

Keohane, Clodagh; Mesa, Ruben; Harrison, Claire

2013-01-01

In 2005, the description of the JAK2V617F mutation for the first time provided a molecular key to enable more rapid diagnosis and target for novel therapeutics in the myeloproliferative neoplasms. In 2007, the first-in-class agent INC18424, ruxolitinib, JAKafi, or JAKAVI was first tested in patients with intermediate-risk 2 or high-risk myelofibrosis regardless of whether they possessed the JAK2V617F mutation. Patients treated with this agent had major reduction in splenomegaly as well as impressive reduction, and in some cases resolution, of symptoms. This study was followed by the two Controlled Myelofibrosis Study with Oral JAK Inhibitor Therapy (COMFORT) trials (the first-ever phase III trials in myelofibrosis), which confirmed results in these aspects were superior to either placebo or standard care, and updated results show a survival advantage with this therapy. This paper discusses these results and data from other JAK inhibitors while speculating on the future of these therapies. It also reflects on the fact that the true targets and agents' mode of action are uncertain. Unlike targeted therapy for chronic myeloid leukemia (CML), these agents do not deliver molecular remission, and it is not clear whether their predominant benefit is mediated via JAK2, JAK1, or both. Nonetheless, the advent of the JAK inhibitor is a welcome advance and has made a dramatic improvement to the therapeutic landscape of these conditions.

Successfully treated transoral crossbow injury to the axial spine causing mild neurologic deficit: case presentation.

PubMed

Kovari, Viktor Zsolt

2017-05-01

To detail the management, complications and results of a crossbow arrow injury, where the broadhead went through the mouth, tongue, soft palate, C2 vertebra, spinal canal, dural sack, exiting the neck posteriorly and the arrow shaft lodged in the spine causing mild spinal cord injury. Case presentation. A penetrating axial cervical spine crossbow injury was treated successfully in spite of the following interdisciplinary complications: meningitis, cerebrospinal fluid leakage, re-bleeding, and cardiac arrest. The shaft was removed from the neck, and C1-3 dorsal stabilization was performed. Controlled Computed Tomography (CT) showed adequate implant position. After 4 months the patient's fine motor skills improved, and he became able to button his shirt on his own, and to eat and drink without any help. Additionally, he was able to walk without any support. At the time of control at the outpatient clinic his behavior was adequate: he cooperated with the examining doctor and answered with short sentences although his psychomotor skills were slightly slower. Although bow and crossbow spine injuries are rare nowadays they still occur. The removal of a penetrating missile resulting in such a spinal injury required a unique solution. General considerations, such as securing the airway, leaving the penetrating arrow in the neck and immobilizing both the arrow and neck for transport, thorough diagnostic imaging, preventing cerebrospinal fluid leakage, administering prophylactic antibiotics with broad coverage and stabilizing the spine if required, are advised.

Relapse of Takayasu arteritis as a cause of suicidal poisoning and subsequent major ischemic stroke successfully treated with thrombolytic therapy.

PubMed

Komatina, Neneal; Lepić, Toplica; Labović, Boban; Stevović, Tatjana; Petronijević, Milan; Radovinović-Tasić, Sanja; Obradović, Dragana

2016-08-01

Takayasu arteritis (TA) is a rare large vessel arteritis, affecting primarily aorta and its major branches. Its clinical manifestations can vary significantly - from asymptomatic to serious vascular events. Acute neurological complications are frequent at the onset of the disease and in relapses. Anxiety and depression are more frequent in TA patients than in general population as well as during relapses. Prevalence of transient ischemic attack or ischemic stroke in TA patients is approximately 10-20%. We presented a patient with TA that began with a depressive episode resulting in attempted suicide by bromazepame poisoning. This was subsequently followed by major ischemic stroke caused by thrombosis of the left middle cerebral artery (probably due to aortic arch embolism) successfully treated with intravenous thrombolysis. Intravenous thrombolysis appears to be safe and effective in patients with TA and stroke.

Results of a phase 2 study of pacritinib (SB1518), a JAK2/JAK2(V617F) inhibitor, in patients with myelofibrosis

PubMed Central

Seymour, John F.; Roberts, Andrew W.; Wadleigh, Martha; To, L. Bik; Scherber, Robyn; Turba, Elyce; Dorr, Andrew; Zhu, Joy; Wang, Lixia; Granston, Tanya; Campbell, Mary S.; Mesa, Ruben A.

2015-01-01

Pacritinib (SB1518) is a Janus kinase 2 (JAK2), JAK2(V617F), and Fms-like tyrosine kinase 3 inhibitor that does not inhibit JAK1. It demonstrated a favorable safety profile with promising efficacy in phase 1 studies in patients with primary and secondary myelofibrosis (MF). This multicenter phase 2 study further characterized the safety and efficacy of pacritinib in the treatment of patients with MF. Eligible patients had clinical splenomegaly poorly controlled with standard therapies or were newly diagnosed with intermediate- or high-risk Lille score. Patients with any degree of cytopenia were eligible. Thirty-five patients were enrolled. At entry, 40% had hemoglobin <10 g/dL and 43% had platelets <100 000× 109/L. Up to week 24, 8 of 26 evaluable patients (31%) achieved a ≥35% decrease in spleen volume determined by magnetic resonance imaging and 14 of 33 (42%) attained a ≥50% reduction in spleen size by physical examination. Median MF symptom improvement was ≥50% for all symptoms except fatigue. Grade 1 or 2 diarrhea (69%) and nausea (49%) were the most common treatment-emergent adverse events. The study drug was discontinued in 9 patients (26%) due to adverse events (4 severe). Pacritinib is an active agent in patients with MF, offering a potential treatment option for patients with preexisting anemia and thrombocytopenia. This trial was registered at www.clinicaltrials.gov as #NCT00745550. PMID:25762180

miRNA-mRNA integrative analysis in primary myelofibrosis CD34+ cells: role of miR-155/JARID2 axis in abnormal megakaryopoiesis

PubMed Central

Norfo, Ruggiero; Zini, Roberta; Pennucci, Valentina; Bianchi, Elisa; Salati, Simona; Guglielmelli, Paola; Bogani, Costanza; Fanelli, Tiziana; Mannarelli, Carmela; Rosti, Vittorio; Pietra, Daniela; Salmoiraghi, Silvia; Bisognin, Andrea; Ruberti, Samantha; Rontauroli, Sebastiano; Sacchi, Giorgia; Prudente, Zelia; Barosi, Giovanni; Cazzola, Mario; Rambaldi, Alessandro; Bortoluzzi, Stefania; Ferrari, Sergio; Tagliafico, Enrico; Vannucchi, Alessandro M.

2014-01-01

Primary myelofibrosis (PMF) is a myeloproliferative neoplasm characterized by megakaryocyte (MK) hyperplasia, bone marrow fibrosis, and abnormal stem cell trafficking. PMF may be associated with somatic mutations in JAK2, MPL, or CALR. Previous studies have shown that abnormal MKs play a central role in the pathophysiology of PMF. In this work, we studied both gene and microRNA (miRNA) expression profiles in CD34+ cells from PMF patients. We identified several biomarkers and putative molecular targets such as FGR, LCN2, and OLFM4. By means of miRNA-gene expression integrative analysis, we found different regulatory networks involved in the dysregulation of transcriptional control and chromatin remodeling. In particular, we identified a network gathering several miRNAs with oncogenic potential (eg, miR-155-5p) and targeted genes whose abnormal function has been previously associated with myeloid neoplasms, including JARID2, NR4A3, CDC42, and HMGB3. Because the validation of miRNA-target interactions unveiled JARID2/miR-155-5p as the strongest relationship in the network, we studied the function of this axis in normal and PMF CD34+ cells. We showed that JARID2 downregulation mediated by miR-155-5p overexpression leads to increased in vitro formation of CD41+ MK precursors. These findings suggest that overexpression of miR-155-5p and the resulting downregulation of JARID2 may contribute to MK hyperplasia in PMF. PMID:25097177

Pediatric Case of Xanthogranuloma in the Sellar Region Presenting a Visual Disturbance Successfully Treated with Endoscopic Endonasal Surgery.

PubMed

Nishimura, Fumihiko; Park, Young-Soo; Motoyama, Yasushi; Nakagawa, Ichiro; Yamada, Shuichi; Nakase, Hiroyuki

2018-06-01

Xanthomatous pituitary diseases rarely occur in childhood. We report a rare pediatric case of a xanthogranuloma that developed in the sellar region, resulting in a visual disturbance that was treated successfully with endoscopic endonasal surgery. A 13-year-old boy came to us with a headache and visual disturbance that occurred 1 month prior. Clinical examination findings showed that he was alert with signs of bitemporal hemianopsia. An endocrinologic examination showed partial hypopituitarism, and brain magnetic resonance imaging revealed a cystic mass in the sellar turcica compressing the optic apparatus. Endoscopic endonasal surgery was performed to decompress the optic apparatus, and the mass was removed. Histopathologic analysis of the tumor demonstrated granulomatous tissue with cholesterol clefts, foamy macrophages, and multinucleated giant cells, with no epithelial component. The diagnosis was xanthogranuloma of the sellar region. The patient gradually recovered from the visual disturbance and was free from any neurologic signs or symptoms 6 months after surgery. Xanthogranuloma, although rare, should be considered as a differential diagnosis of a sellar or suprasellar lesion, even in children. Copyright © 2018 Elsevier Inc. All rights reserved.

[Prognostic value of JAK2, MPL and CALR mutations in Chinese patients with primary myelofibrosis].

PubMed

Xu, Z F; Li, B; Liu, J Q; Li, Y; Ai, X F; Zhang, P H; Qin, T J; Zhang, Y; Wang, J Y; Xu, J Q; Zhang, H L; Fang, L W; Pan, L J; Hu, N B; Qu, S Q; Xiao, Z J

2016-07-01

To evaluate the prognostic value of JAK2, MPL and CALR mutations in Chinese patients with primary myelofibrosis (PMF). Four hundred and two Chinese patients with PMF were retrospectively analyzed. The Kaplan-Meier method, the Log-rank test, the likelihood ratio test and the Cox proportional hazards regression model were used to evaluate the prognostic scoring system. This cohort of patients included 209 males and 193 females with a median age of 55 years (range: 15- 89). JAK2V617F mutations were detected in 189 subjects (47.0% ), MPLW515 mutations in 13 (3.2%) and CALR mutations in 81 (20.1%) [There were 30 (37.0%) type-1, 48 (59.3%) type-2 and 3 (3.7%) less common CALR mutations], respectively. 119 subjects (29.6%) had no detectable mutation in JAK2, MPL or CALR. Univariate analysis indicated that patients with CALR type-2 mutations or no detectable mutations had inferior survival compared to those with JAK2, MPL or CALR type- 1 or other less common CALR mutations (the median survival was 74vs 168 months, respectively [HR 2.990 (95% CI 1.935-4.619),P<0.001]. Therefore, patients were categorized into the high-risk with CALR type- 2 mutations or no detectable driver mutations and the low- risk without aforementioned mutations status. The DIPSS-Chinese molecular prognostic model was proposed by adopting mutation categories and DIPSS-Chinese risk group. The median survival of patients classified in low risk (132 subjects, 32.8% ), intermediate- 1 risk (143 subjects, 35.6%), intermediate- 2 risk (106 subjects, 26.4%) and high risk (21 subjects, 5.2%) were not reached, 156 (95% CI 117- 194), 60 (95% CI 28- 91) and 22 (95% CI 10- 33) months, respectively, and there was a statistically significant difference in overall survival among the four risk groups (P<0.001). There was significantly higher predictive power for survival according to the DIPSS-Chinese molecular prognostic model compared with the DIPSS-Chinese model (P=0.005, -2 log-likelihood ratios of 855.6 and 869

Chronic active Epstein-Barr virus infection with mosquito allergy successfully treated with reduced-intensity unrelated allogeneic bone marrow transplantation in a boy.

PubMed

Matsunaga, Takayuki; Kurosawa, Hidemitsu; Okuya, Mayuko; Nakajima, Daisuke; Hagisawa, Susumu; Sato, Yuya; Fukushima, Keitaro; Sugita, Kenichi; Arisaka, Osamu

2009-03-01

EBV-infected T-/NK cells play an important role in the pathogenesis of mosquito allergy, and the prognosis of most patients with mosquito allergy is poor without proper treatment. We describe a 13-yr-old boy who had CAEBV with mosquito allergy and was successfully treated with BMT from an unrelated donor after reduced-intensity preconditioning. Because combination chemotherapy failed to achieve CR, we performed unrelated BMT to reconstitute normal immunity and eradicate any residual EBV-infected cells. To reduce complications after BMT, we selected a reduced-intensity preconditioning regimen consisting of fludarabine, l-phenylalanine mustard, and antithymocyte Ig instead of a conventional myeloablative preconditioning. Although grade II acute GVHD developed, it was successfully controlled with immunosuppressive therapy. After 27 months, the patient has been well without any signs of CAEBV, and the EBV DNA has been undetectable with real-time PCR analysis. We conclude that RIST from the bone marrow of an unrelated donor is indicated for some patients who have CAEBV that is refractory to chemotherapy and who have no HLA-matched related donors or cord blood as a source of stem cells.

Tools for Success in Private Practice

PubMed Central

Kasewurm, Gyl

2016-01-01

When it comes to business, there is no one thing that ensures success. However, focusing on helping as many patients as possible and tracking those results, creating a program to gather referrals from your own patients, setting a goal each month, and having a key employee to guide operations at the front desk to ensure that every patient is treated well and has an awesome experience at your office will go a long way to steering a practice to success. PMID:28028328

Tools for Success in Private Practice.

PubMed

Kasewurm, Gyl

2016-11-01

When it comes to business, there is no one thing that ensures success. However, focusing on helping as many patients as possible and tracking those results, creating a program to gather referrals from your own patients, setting a goal each month, and having a key employee to guide operations at the front desk to ensure that every patient is treated well and has an awesome experience at your office will go a long way to steering a practice to success.

Total Parenteral Nutrition Successfully Treating Black Esophagus Secondary to Hypovolemic Shock.

PubMed

Brar, Tony S; Helton, Richard; Zaidi, Zareen

2017-01-01

We present a patient who developed black esophagus secondary to hypovolemic shock and was placed on total parenteral nutrition for three weeks after hospital discharge. The area of interest is the multimodal approach used in treatment of this noncompliant patient. Even with a high mortality rate, this case illustrates a successful outcome of a patient who responded to appropriate immediate therapy resulting in complete resolution of the necrosis with no further development of complications.

MPLW515L mutation in acute megakaryoblastic leukaemia.

PubMed

Hussein, K; Bock, O; Theophile, K; Schulz-Bischof, K; Porwit, A; Schlue, J; Jonigk, D; Kreipe, H

2009-05-01

The thrombopoietin receptor gene (MPL) is expressed in megakaryocytes and exhibits the gain of function point mutation W515K/L in approximately 5% of patients with primary myelofibrosis/idiopathic myelofibrosis (PMF) representing one subtype of the chronic myeloproliferative disorders (myeloproliferative neoplasm). A series of primary and secondary acute myeloid leukaemias (AML) with megakaryoblastic phenotype and myelofibrosis unrelated to PMF (n=12) was analysed for the MPL(W515K/L) mutation by pyrosequencing. In three cases (25%), MPL(W515L) was found and in two of these a combination with trisomy 21 or the Philadelphia chromosome occurred. None of the secondary AML cases evolving from pre-existing PMF showed MPL(W515K/L) (n=4). We conclude that MPL(W515L) occurs in a considerable proportion of acute megakaryoblastic leukaemias with myelofibrosis unrelated to PMF.

Total Parenteral Nutrition Successfully Treating Black Esophagus Secondary to Hypovolemic Shock

PubMed Central

Helton, Richard; Zaidi, Zareen

2017-01-01

We present a patient who developed black esophagus secondary to hypovolemic shock and was placed on total parenteral nutrition for three weeks after hospital discharge. The area of interest is the multimodal approach used in treatment of this noncompliant patient. Even with a high mortality rate, this case illustrates a successful outcome of a patient who responded to appropriate immediate therapy resulting in complete resolution of the necrosis with no further development of complications. PMID:28702267

Fludarabine Phosphate, Melphalan, and Low-Dose Total-Body Irradiation Followed by Donor Peripheral Blood Stem Cell Transplant in Treating Patients With Hematologic Malignancies

ClinicalTrials.gov

2017-09-08

Accelerated Phase Chronic Myelogenous Leukemia; Adult Acute Lymphoblastic Leukemia in Remission; Adult Acute Myeloid Leukemia in Remission; Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); Adult Grade III Lymphomatoid Granulomatosis; Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Aplastic Anemia; Burkitt Lymphoma; Childhood Acute Lymphoblastic Leukemia in Remission; Childhood Acute Myeloid Leukemia in Remission; Childhood Chronic Myelogenous Leukemia; Childhood Diffuse Large Cell Lymphoma; Childhood Grade III Lymphomatoid Granulomatosis; Childhood Immunoblastic Large Cell Lymphoma; Childhood Myelodysplastic Syndromes; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Chronic Myelomonocytic Leukemia; Chronic Phase Chronic Myelogenous Leukemia; Congenital Amegakaryocytic Thrombocytopenia; Diamond-Blackfan Anemia; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Juvenile Myelomonocytic Leukemia; Myelodysplastic/Myeloproliferative Neoplasm, Unclassifiable; Nodal Marginal Zone B-cell Lymphoma; Paroxysmal Nocturnal Hemoglobinuria; Peripheral T-cell Lymphoma; Polycythemia Vera; Post-transplant Lymphoproliferative Disorder; Previously Treated Myelodysplastic Syndromes; Primary Myelofibrosis; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell

Early Uncomplicated Appendicitis-Who Can We Treat Nonoperatively?

PubMed

Horattas, Mark C; Horattas, Ileana K; Vasiliou, Elya M

2018-02-01

This study evaluated nonoperative treatment for mild appendicitis and reviewed selection criteria to be used in introducing this option into clinical practice. A retrospective review of 73 consecutive cases of appendicitis treated by a single surgeon from 2011 to 2013 was completed. Patients who were diagnosed with mild appendicitis meeting the criteria of an APPENDICITIS scoring algorithm proposed in this manuscript were considered for nonoperative management. An additional 17 patients with mild appendicitis were offered and successfully treated nonoperatively between 2014 and 2016 and reviewed. Of these original 73 patients, 37 had moderate to severe appendicitis and directly underwent appendectomy. The remaining patients were diagnosed with mild appendicitis and considered eligible for nonoperative management. Of these, 14 patients were offered nonoperative therapy. Thirteen responded successfully; one patient responded partially, but later opted for surgery. In 2014, this scoring system and preliminary results were shared with the other surgeons in our department. Nonoperative management was then selectively adopted by a few of the surgeons from 2014 to 2016 with another 17 patients (APPENDICITIS score of 0 or 1) being offered and successfully managed nonoperatively. Patients with mild or early appendicitis can be successfully managed nonoperatively. A proposed APPENDICITIS scoring system may provide a helpful mnemonic for successfully selecting patients for this option.

ON012380: A Non-ATP Competitive Inhibitor of BCR-ABL for the Therapy of Imatinib-Resistant CMLs

DTIC Science & Technology

2007-05-01

polycythemia vera (PV) as well as in some cases of essential thrombocythemia (ET) and chronic idiopathic myelofibrosis (CIMF), all of which are...in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis. Cancer Cell. 7(4):387-97. 20 Meydan N, Grunberger T

Histoplasmosis treated with a sulphonamide. A case report.

PubMed

Mackenjee, M K; Coovadia, H M

1976-11-20

Disseminated histoplasmosis involving the reticuloendothelial system in a Black boy, successfully treated with a sulphonamide, is reported. The importance of this rare disease in South Africa is discussed.

Life for patients with myelofibrosis: the physical, emotional and financial impact, collected using narrative medicine-Results from the Italian 'Back to Life' project.

PubMed

Palandri, Francesca; Benevolo, Giulia; Iurlo, Alessandra; Abruzzese, Elisabetta; Carella, Angelo M; Paoli, Chiara; Palumbo, Giuseppe A; Bonifacio, Massimiliano; Cilloni, Daniela; Andriani, Alessandro; Guarini, Attilio; Turri, Diamante; Elli, Elena Maria; Falcone, Antonietta; Anaclerico, Barbara; Musto, Pellegrino; Di Renzo, Nicola; Tiribelli, Mario; Zambello, Renato; Spinosa, Caterina; Ricco, Alessandra; Raucci, Letizia; Martino, Bruno; Annunziata, Mario; Pascale, Silvia; Liberati, Anna Marina; La Nasa, Giorgio; Maffioli, Margherita; Breccia, Massimo; Pugliese, Novella; Betti, Silvia; Giglio, Gianfranco; Cappuccio, Antonietta; Reale, Luigi

2018-06-01

Myelofibrosis (MF) is a chronic myeloproliferative neoplasm characterised by an aggressive clinical course, with disabling symptoms and reduced survival. Patients experience a severely impaired quality of life and their families face the upheaval of daily routines and high disease-related financial costs. The aim of this study was to investigate the perceptions of Italian patients and their caregivers about living with MF and the burden of illness associated with MF. A quali-quantitative questionnaire and a prompted written narrative survey were administered to patients affected by primary or post-essential thrombocythemia/post-polycythaemia vera MF and their primary caregiver in 35 Italian haematological centres. In total, 287 questionnaires were returned by patients and 98 by caregivers, with 215 and 62, respectively, including the narrative. At the time of diagnosis, the most commonly expressed emotional states of patients were fear, distress and anger, confirming the difficulty of this phase. A high level of emotional distress was also reported by caregivers. Along the pathway of care, the ability to cope with the disease differed according to the quality of care received. The mean cost to each patient attributable to MF was estimated as €12,466 per year, with an estimated average annual cost of loss of income of €7774 per patient and €4692 per caregiver. Better understanding of the personal life of MF patients and their families could improve the relationships between health workers and patients, resulting in better focused healthcare pathways and more effective financial support to maintain patients in their social roles.

Primary diffuse large B cell lymphoma of the uterine cervix successfully treated by combined chemotherapy alone: A case report.

PubMed

Cubo, Ana María; Soto, Zandra Mileny; Cruz, Miguel Ángel; Doyague, María José; Sancho, Verónica; Fraino, Aurymar; Blanco, Óscar; Puig, Noemi; Alcoceba, Miguel; González, Marcos; Sayagués, José María

2017-05-01

Primary lymphomas of the uterine cervix are a rare disease. They are often misdiagnosed because of their rarity and because they can be easily confused with a squamous cell carcinoma of the cervix, as they are usually presented as exophytic mass with vaginal bleeding as their most common symptoms. Nevertheless, considering that both the prognosis and the treatment are completely different between them, differential diagnosis should be taken into account. A case of a 51-year-old woman with a primary diffuse large B-cell lymphoma of the cervix is presented. Diagnosis of this tumor was a challenge for pathologists and clinicians, as four biopsies were needed to achieve a final diagnosis. Patient was successfully treated with combined Rituximab and chemotherapy (R-CHOP) alone. Complete remission, confirmed through biopsy, was reached after six courses of chemotherapy. At 2-years follow up, patient is alive and free of disease. Considering that the prognosis and treatment of primary malignant lymphoma of the cervix are completely different than that of the squamous cell carcinoma, awareness of this disease should be considered in the differential diagnosis.

Autotransplantation of endodontically treated third molars.

PubMed

Sobhi, Muhammad Bakhsh; Rana, Muzammil Jamil Ahmed; Manzoor, Manzoor Ahmed; Ibrahim, Mohammad; Tasleem-ul-Hudda

2003-07-01

To determine the success rate in autotransplantation of endodontically treated third molars. A descriptive study. This study was carried out at Armed Forces Institute of Dentistry (AFID), Rawalpindi (Pakistan) from January 2002 to December 2002. A total of 50 patients meeting the inclusion criteria were selected who had their first or second molars in unrestorable condition with intact third molars. The donor teeth were extracted after the preparation of recipient site. After endodontically treated in vitro the donor teeth were carried to the recipient site and immobilized. Postoperative variables were recorded and analyzed on SPSS version 10. The overall success rate after six months of the transplantation of third molars was 88% with complete root formation after endodontic treatment. All the patients(12%) who had complaints were more than 35 years of age. Third molars are good substitute for the unrestorable first or second molars and would be as effective as endosseous implants. The procedure is likely to have complications in the advanced age group.

Histologic and cytologic bone marrow findings in dogs with suspected precursor-targeted immune-mediated anemia and associated phagocytosis of erythroid precursors.

PubMed

Lucidi, Cynthia de A; de Rezende, Christian L E; Jutkowitz, L Ari; Scott, Michael A

2017-09-01

Precursor-targeted immune-mediated anemia (PIMA) has been suspected in dogs with nonregenerative anemia and bone marrow findings varying from erythroid hyperplasia to pure red cell aplasia. Phagocytosis of erythroid precursors/rubriphagocytosis (RP) reported in some affected dogs suggests a destructive component to the pathogenesis of PIMA. The purpose of the study was to characterize laboratory and clinical findings in dogs with suspected PIMA and RP, with emphasis on cytologic and histologic bone marrow findings. Dogs with PIMA and RP were identified by review of paired bone marrow aspirate and core biopsy slides collected over a 4-year period. Samples were systematically assessed and characterized along with other pertinent laboratory data and clinical findings. Twenty-five dogs met criteria for PIMA and had RP that was relatively stage-selective. Erythropoiesis was expanded to the stage of erythroid precursors undergoing most prominent phagocytosis, yielding patterns characterized by a hypo-, normo-, or hypercellular erythroid lineage. A 4 th pattern involved severe collagen myelofibrosis, and there was a spectrum of mild to severe collagen myelofibrosis overall. Evidence of immune-mediated hemolysis was rare. Immunosuppressive therapy was associated with remission in 77% of dogs treated for at least the median response time of 2 months. Bone marrow patterns in dogs fulfilling criteria for PIMA were aligned with stage-selective phagocytosis of erythroid precursors and the development of collagen myelofibrosis, common in dogs with PIMA. Recognition of these patterns and detection of RP facilitates diagnosis of PIMA, and slow response to immunosuppressive therapy warrants further investigation into its pathogenesis. © 2017 American Society for Veterinary Clinical Pathology.

Combining Drugs to Treat Ovarian Cancer - Annual Plan

Cancer.gov

Approximately 70 percent of women diagnosed with ovarian cancer will die from the disease. Read about the NCI-funded combination drug trial that has successfully treated Betsy Brauser's recurrent cancer.

Management of Platelet Transfusion Therapy in Patients With Blood Cancer or Treatment-Induced Thrombocytopenia

ClinicalTrials.gov

2018-02-15

Acute Biphenotypic Leukemia; Acute Lymphoblastic Leukemia; Acute Myeloid Leukemia; B-Cell Non-Hodgkin Lymphoma; Chronic Lymphocytic Leukemia; Chronic Myelogenous Leukemia, BCR-ABL1 Positive; Hematologic and Lymphocytic Disorder; Hematopoietic Cell Transplantation Recipient; Myelodysplastic Syndrome; Primary Myelofibrosis; Secondary Myelofibrosis; T-Cell Non-Hodgkin Lymphoma; Thrombocytopenia; Venous Thromboembolism

How I treat polycythemia vera.

PubMed

Passamonti, Francesco

2012-07-12

Polycythemia vera (PV) is a clonal disorder characterized by unwarranted production of red blood cells. In the majority of cases, PV is driven by oncogenic mutations that constitutively activate the JAK-STAT signal transduction pathway, such as JAK2 V617F, or exon 12 mutations or LNK mutations. Diagnosis of PV is based on the WHO criteria. Diagnosis of post-PV myelofibrosis is established according to the International Working Group for Myeloproliferative Neoplasms Research and Treatment criteria. Different clinical presentations of PV are discussed. Prognostication of PV is tailored to the most frequent complication during follow-up, namely, thrombosis. Age older than 60 years and prior history of thrombosis are the 2 main risk factors for disease stratification. Correlations are emerging between leukocytosis, JAK2(V617F) mutation, BM fibrosis, and different outcomes of PV, which need to be confirmed in prospective studies. In my practice, hydroxyurea is still the "gold standard" when cytoreduction is needed, even though pegylated IFN-alfa-2a and ruxolitinib might be useful in particular settings. Results of phase 1 or 2 studies concerning these latter agents should however be confirmed by the ongoing randomized phase 3 clinical trials. In this paper, I discuss the main problems encountered in daily clinical practice with PV patients regarding diagnosis, prognostication, and therapy.

A case of allergic bronchopulmonary aspergillosis successfully treated with mepolizumab.

PubMed

Terashima, Takeshi; Shinozaki, Taro; Iwami, Eri; Nakajima, Takahiro; Matsuzaki, Tatsu

2018-03-27

Allergic bronchopulmonary aspergillosis (ABPA) is an allergic pulmonary disease comprising a complex hypersensitivity reaction to Aspergillus fumigatus. Clinical features of ABPA are wheezing, mucoid impaction, and pulmonary infiltrates. Oral corticosteroids and anti-fungal agents are standard therapy for ABPA, but long-term use of systemic corticosteroids often causes serious side effects. A 64-year-old woman was diagnosed with ABPA based on a history of bronchial asthma (from 40 years of age), elevated total IgE, the presence of serum precipitating antibodies and elevated specific IgE antibody to A. fumigatus, and pulmonary infiltration. Bronchoscopy showed eosinophilic mucoid impaction. Systemic corticosteroid therapy was initiated, and her symptoms disappeared. Peripheral eosinophilia and pulmonary infiltration recurred five months after cessation of corticosteroid treatment. Systemic corticosteroids were re-initiated and itraconazole was added as an anti-fungal agent. The patient was free of corticosteroids, aside from treatment with a short course of systemic corticosteroids for asthma exacerbation, and clinically stable with itraconazole and asthma treatments for 3 years. In 2017, she experienced significant deterioration. Laboratory examination revealed marked eosinophilia (3017/μL) and a chest computed tomography (CT) scan demonstrated pulmonary infiltration in the left upper lobe and mucoid impaction in both lower lobes. The patient was treated with high-dose inhaled corticosteroid/long-acting beta-agonist, a long-acting muscarinic antagonist, a leukotriene receptor antagonist, and theophylline; spirometry revealed a forced expiratory volume in 1 s (FEV 1 ) of 1.01 L. An uncontrolled asthma state was indicated by an Asthma Control Test (ACT) score of 18. Mepolizumab, 100 mg every 4 weeks, was initiated for the treatment of severe bronchial asthma with ABPA exacerbation. Bronchial asthma symptoms dramatically improved, and ACT score increased to 24

Retrospective analysis of factors that affect the success of single-dose methotrexate treatment in ectopic pregnancy

PubMed Central

Var, Altan; Özyurt, Ramazan; Şık, Bulat Aytek; Kumbasar, Serkan; Sever, Erman; Deveci, Mustafa; Çöt, Özgür; Salman, Süleyman; Güzel, Yılmaz

2015-01-01

Objective: Detection of factors that affect the success of single-dose methotrexate treatment in ectopic pregnancy. Materials and Methods: We investigated 99 patients who had been treated with single-dose methotrexate for ectopic pregnancy in our clinic between January 2009 and June 2014. Demographic, clinical, and laboratory results of possible factors that affect treatment success were retrospectively analyzed. Successfully and unsuccessfully treated patients were compared based on their pre-treatment results. Results: The success rate of single-dose methotrexate treatment was found to be 70.7%. No significant difference was found between succesfully and unsuccessfully treated patients before treatment in terms of factors such as gestational weeks, mass size, presence of yolk sac, and presence of free fluid (p=0.224, p=0.201, p=0.200, p=0.200). Serum β-hCG values in patients whose treatment was unsuccessful was found to be higher compared with the successfully treated group (mean β-hCG value of unsuccessful group: 4412±3501 mIU/mL; mean β-hCG value of successful group: 1079±942 mIU/mL; p<0.001). Conclusion: Single-dose methotrexate treatment is an effective and reliable method in the treatment of ectopic pregnancy. Elevation of serum β-hCG value stands as the main prognostic factor that affects the success of single-dose methotrexate treatment. PMID:28913072

Umbilical Cord Blood Transplant, Cyclophosphamide, Fludarabine Phosphate, and Total-Body Irradiation in Treating Patients With Hematologic Disease

ClinicalTrials.gov

2018-03-23

Acute Biphenotypic Leukemia; Acute Myeloid Leukemia Arising From Previous Myelodysplastic Syndrome; Acute Myeloid Leukemia in Remission; Adult Acute Lymphoblastic Leukemia in Complete Remission; Aggressive Non-Hodgkin Lymphoma; Beta-2-Microglobulin Greater Than 3 g/mL; Blasts Under 5 Percent of Bone Marrow Nucleated Cells; Burkitt Lymphoma; Childhood Acute Lymphoblastic Leukemia in Complete Remission; Chromosome 13 Abnormality; Chronic Phase Chronic Myelogenous Leukemia, BCR-ABL1 Positive; Lymphoblastic Lymphoma; Mantle Cell Lymphoma; Myelodysplastic Syndrome With Excess Blasts; Myelofibrosis; Pancytopenia; Plasma Cell Myeloma; Prolymphocytic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Recurrent Chronic Lymphocytic Leukemia; Recurrent Chronic Myelogenous Leukemia, BCR-ABL1 Positive; Recurrent Follicular Lymphoma; Recurrent Lymphoplasmacytic Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Small Lymphocytic Lymphoma

Establishment and Early Succession of Bacterial Communities in Monochloramine-Treated Drinking Water Biofilms

EPA Science Inventory

Monochloramine is increasingly used as a drinking water disinfectant because it forms lower levels of regulated disinfection by-products. While its use has been shown to increase nitrifying bacteria, little is known about the bacterial succession within biofilms in monochloramin...

Making see and treat work for patients and staff.

PubMed

Parker, Louise

2004-02-01

Every department is at a different stage in the development of see and treat. Teams have been established in various ways and are experiencing different dilemmas in making see and treat work best. It is not enough to pick up an established see and treat model, place it in an emergency department and sit back and watch the results. There is no 'magic wand'; no single determining factor to make see and treat work well. Influencing factors need to be understood, applied locally and reviewed regularly to assess success. The NHS Modernisation Agency publishes its survey report, See and Treat: Making it work for patients and staff, on February 4. For Further details, access www.modern.nhs.uk/emergency

JAK2 mutations and clinical practice in myeloproliferative neoplasms.

PubMed

Tefferi, Ayalew

2007-01-01

With the discovery in the last 3 years of novel Janus kinase 2 (JAK2) and thrombopoietin receptor (MPL) mutations, the pathogenetic understanding of and clinical practice for myeloproliferative neoplasms (MPNs) have entered a new era. Each one of these newly discovered mutations, including JAK2V617F, MPLW515L, and a JAK2 exon 12 mutation, has been shown to result in constitutive activation of JAK-STAT signaling and also induce a MPN phenotype in mice. Thus, JAK2 is now considered to be a legitimate target for drug development in MPNs, and small molecule JAK2 inhibitors have already gone through successful preclinical testing, and early-phase human trials in primary myelofibrosis have already begun. Furthermore, JAK2 mutation screening has now become a front-line diagnostic test in the evaluation of both "erythrocytosis" and thrombocytosis and the 2001 World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis have now been revised to incorporate JAK2V617F mutation screening.

Haematological manifestations of lupus

PubMed Central

Fayyaz, Anum; Igoe, Ann; Kurien, Biji T; Danda, Debashish; James, Judith A; Stafford, Haraldine A; Scofield, R Hal

2015-01-01

Our purpose was to compile information on the haematological manifestations of systemic lupus erythematosus (SLE), namely leucopenia, lymphopenia, thrombocytopenia, autoimmune haemolytic anaemia (AIHA), thrombotic thrombocytopenic purpura (TTP) and myelofibrosis. During our search of the English-language MEDLINE sources, we did not place a date-of-publication constraint. Hence, we have reviewed previous as well as most recent studies with the subject heading SLE in combination with each manifestation. Neutropenia can lead to morbidity and mortality from increased susceptibility to infection. Severe neutropenia can be successfully treated with granulocyte colony-stimulating factor. While related to disease activity, there is no specific therapy for lymphopenia. Severe lymphopenia may require the use of prophylactic therapy to prevent select opportunistic infections. Isolated idiopathic thrombocytopenic purpura maybe the first manifestation of SLE by months or even years. Some manifestations of lupus occur more frequently in association with low platelet count in these patients, for example, neuropsychiatric manifestation, haemolytic anaemia, the antiphospholipid syndrome and renal disease. Thrombocytopenia can be regarded as an important prognostic indicator of survival in patients with SLE. Medical, surgical and biological treatment modalities are reviewed for this manifestation. First-line therapy remains glucocorticoids. Through our review, we conclude glucocorticoids do produce a response in majority of patients initially, but sustained response to therapy is unlikely. Glucocorticoids are used as first-line therapy in patients with SLE with AIHA, but there is no conclusive evidence to guide second-line therapy. Rituximab is promising in refractory and non-responding AIHA. TTP is not recognised as a criteria for classification of SLE, but there is a considerable overlap between the presenting features of TTP and SLE, and a few patients with SLE have concurrent

Asparaginase-associated concurrence of hyperlipidemia, hyperglobulinemia, and thrombocytosis was successfully treated by centrifuge/membrane hybrid double-filtration plasmapheresis.

PubMed

Wang, Taina; Xu, Bin; Fan, Rong; Liu, Zhihong; Gong, Dehua

2016-01-01

Asparaginase-associated concurrence of hyperlipidemia, hyperglobulinemia, and thrombocytosis is a rare complication requiring aggressive lipoprotein apheresis, but no one of currently available lipoprotein apheresis methods can simultaneously resolve the 3 abnormalities. Herein, we reported a construction of double-filtration plasmapheresis (DFPP) using a combination of centrifugal/membranous plasma separation techniques to successfully treat a patient with hyperlipidemia, hyperglobulinemia, and thrombocytosis. A male presented with severe hyperlipidemia, hyperglobulinemia, and thrombocytosis during asparaginase treatment for NK/T-cell lymphoblastic lymphoma and was scheduled to receive lipoprotein apheresis. To simultaneously remove lipoproteins, immunoglobulin, and deplete platelets from blood, a centrifuge/membrane hybrid DFPP was constructed as following steps: plasma and part of platelets were separated first from whole blood by centrifugal technique and then divided by a fraction plasma separator into 2 parts: platelets and plasma components with large size, which were discarded; and those containing albumin, which were returned to blood with a supplement of extrinsic albumin solution. DFPP lasted 240 minutes uneventfully, processing 5450-mL plasma. The concentrations of plasma components before DFPP were as follows: triglycerides 38.22 mmol/L, total cholesterols 22.98 mmol/L, immunoglobulin A (IgA) 15.7 g/L, IgG 12.7 g/L, and IgM 14.3 g/L; whereas after treatment were 5.69 mmol/L, 2.38 mmol/L, 2.5 g/L, 7.7 g/L, and 0.4 g/L, respectively. The respective reduction ratio was 85.1%, 89.6%, 83.9%, 39.4%, and 96.9%. Platelet count decreased by 40.4% (from 612 × 10(9)/L to 365 × 10(9)/L). Centrifuge/membrane hybrid DFPP can simultaneously remove lipoproteins, immunoglobulin, and deplete platelets, with a success in treatment of asparaginase treatment-induced hyperlipidemia, hyperglobulinemia, and thrombocytosis, and may be useful for patients

Restoration of Endodontically Treated Molars Using All Ceramic Endocrowns

PubMed Central

Carlos, Roopak Bose; Thomas Nainan, Mohan; Pradhan, Shamina; Roshni Sharma; Benjamin, Shiny; Rose, Rajani

2013-01-01

Clinical success of endodontically treated posterior teeth is determined by the postendodontic restoration. Several options have been proposed to restore endodontically treated teeth. Endocrowns represent a conservative and esthetic restorative alternative to full coverage crowns. The preparation consists of a circular equigingival butt-joint margin and central retention cavity into the entire pulp chamber constructing both the crown and the core as a single unit. The case reports discussed here are moderately damaged endodontically treated molars restored using all ceramic endocrowns fabricated using two different systems, namely, CAD/CAM and pressed ceramic. PMID:24455318

Successive construction of cellulase hyperproducers of Trichoderma using hyperpolyploids.

PubMed

Toyama, H; Toyama, N

2000-01-01

When the swollen conidia of Trichoderma reesei QM 6a are treated with 0.1% (w/v) colchicine solution, huge autopolyploid nuclei can be formed in those swollen conidia. When a mycelial mat derived from such a conidum is treated with a haploidizing reagent, benomyl, many fan-shaped sectors are produced from the colony, and cellulase hyperproducers are selected from conidia on the colony. When colchicine and benomyl treatments are repeated on cellulase hyperproducers, new hyperproducers can be constructed successively and systematically. Moreover, when conidia derived from autopolyploids are treated with ethylmethanesulfonate solution, another type of cellulase hyperproducers (polyploids) can be obtained.

Fungal and bacterial community succession differs for three wood types during decay in a forest soil.

PubMed

Prewitt, Lynn; Kang, Youngmin; Kakumanu, Madhavi L; Williams, Mark

2014-08-01

Wood decomposition by soil microorganisms is vital to carbon and nutrient cycles of forested ecosystems. Different wood types decompose at different rates; however, it is not known if there are differences in microbial community succession associated with the decay of different wood types. In this study, the microbial community associated with the decay of pine (decay-susceptible wood), western red cedar (decay resistant) and ACQ-treated pine (Ammoniacal Copper Quaternary, preservative-treated pine for decay resistance) in forest soil was characterized using DNA sequencing, phospholipid fatty acid (PLFA) analysis, and microbial activity over a 26-month period. Bray-Curtis ordination using an internal transcribed spacer (ITS) sequence and PLFA data indicated that fungal communities changed during succession and that wood type altered the pattern of succession. Nondecay fungi decreased over the 26 months of succession; however, by 18 months of decay, there was a major shift in the fungal communities. By this time, Trametes elegans dominated cedar and Phlebia radiata dominated pine and ACQ-treated pine. The description of PLFA associated with ACQ-treated pine resembled cedar more than pine; however, both PLFA and ITS descriptions indicated that fungal communities associated with ACQ-treated pine were less dynamic, perhaps a result of the inhibition by the ACQ preservative, compared with pine and cedar. Overall, fungal community composition and succession were associated with wood type. Further research into the differences in community composition will help to discern their functional importance to wood decay.

A systematic review of implant outcomes in treated periodontitis patients.

PubMed

Sousa, Vanessa; Mardas, Nikos; Farias, Bruna; Petrie, Aviva; Needleman, Ian; Spratt, David; Donos, Nikolaos

2016-07-01

To investigate the effect of treated periodontitis on implant outcomes in partially edentulous individuals compared with periodontally healthy patients. Longitudinal studies reporting on implant survival, success, incidence of peri-implantitis, bone loss and periodontal status, and on partially dentate patients with a history of treated periodontitis were included. The search yielded 14,917 citations. Twenty-seven publications met the inclusion criteria for qualitative data synthesis. Implant success and survival were higher in periodontally healthy patients, whilst bone loss and incidence of peri-implantitis was increased in patients with history of treated periodontitis. There was a higher tendency for implant loss and biological complications in patients previously presenting with severe forms of periodontitis. The strength of the evidence was limited by the heterogeneity of the included studies in terms of study design, population, therapy, unit of analysis, inconsistent definition of baselines and outcomes, as well as by the inadequate reporting of statistical analysis and accounting for confounding factors; thus, meta-analysis could not be performed. Implants placed in patients treated for periodontal disease are associated with higher incidence of biological complications and lower success and survival rates than those placed in periodontally healthy patients. Severe forms of periodontal disease are associated with higher rates of implant loss. However, it is critical to develop well-designed, long-term prospective studies to provide further substantive evidence on the association of these outcomes. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Early Improvement in Marrow Fibrosis Following Haploidentical Stem Cell Transplantation for a Patient with Myelodysplastic Syndrome with Bone Marrow Fibrosis

PubMed Central

Takahashi, Shuichiro; Tsumanuma, Riko; Aizawa, Keiko; Osakabe, Mitsumasa; Maeda, Kunihiko; Omoto, Ejiro

2016-01-01

The prognosis for myelodysplastic syndrome with bone marrow fibrosis (MDS-F) is worse than the prognosis of MDS without fibrosis. Hematopoietic stem cell transplantation (HSCT) is the only curative therapy; however, the indications and the procedures involved in HSCT remain unclear. We herein describe a 69-year-old Japanese man with MDS-F who received haploidentical HSCT and post-transplantation cyclophosphamide. Although the first HSCT resulted in secondary graft failure, the second HSCT using PTCy led to successful engraftment after early improvement in fibrosis. Since the incidence of graft failure is high in myelofibrosis patients, a secondary HSCT using PTCy may be successful if employed. PMID:27853082

Successful management of airway hemangioma with propranolol.

PubMed

Mendiratta, Vibhu; Varghese, Bincy; Chander, Ram; Parakh, Ankit; Solanki, Ravi S

2013-06-01

Airway hemangiomas can be difficult to manage and cause anxiety in both the parents and the treating physician. Propranolol, a nonselective beta-blocker, has recently been used for treating proliferating infantile hemangiomas. We report successful management of a proliferating, large, mixed infantile hemangioma with subglottic extension in an Indian infant using oral propranolol in a dose of 2mg/kg/day without any side effects. Induction of early involution and freedom from the side effects of steroid therapy seem encouraging for using propranolol as a first line treatment modality in the management of troublesome hemangiomas. © 2013 The International Society of Dermatology.

Ruxolitinib vs best available therapy for ET intolerant or resistant to hydroxycarbamide.

PubMed

Harrison, Claire N; Mead, Adam J; Panchal, Anesh; Fox, Sonia; Yap, Christina; Gbandi, Emmanouela; Houlton, Aimee; Alimam, Samah; Ewing, Joanne; Wood, Marion; Chen, Frederick; Coppell, Jason; Panoskaltsis, Nicki; Knapper, Steven; Ali, Sahra; Hamblin, Angela; Scherber, Robyn; Dueck, Amylou C; Cross, Nicholas C P; Mesa, Ruben; McMullin, Mary Frances

2017-10-26

Treatments for high-risk essential thrombocythemia (ET) address thrombocytosis, disease-related symptoms, as well as risks of thrombosis, hemorrhage, transformation to myelofibrosis, and leukemia. Patients resistant/intolerant to hydroxycarbamide (HC) have a poor outlook. MAJIC (ISRCTN61925716) is a randomized phase 2 trial of ruxolitinib (JAK1/2 inhibitor) vs best available therapy (BAT) in ET and polycythemia vera patients resistant or intolerant to HC. Here, findings of MAJIC-ET are reported, where the modified intention-to-treat population included 58 and 52 patients randomized to receive ruxolitinib or BAT, respectively. There was no evidence of improvement in complete response within 1 year reported in 27 (46.6%) patients treated with ruxolitinib vs 23 (44.2%) with BAT ( P = .40). At 2 years, rates of thrombosis, hemorrhage, and transformation were not significantly different; however, some disease-related symptoms improved in patients receiving ruxolitinib relative to BAT. Molecular responses were uncommon; there were 2 complete molecular responses (CMR) and 1 partial molecular response in CALR- positive ruxolitinib-treated patients. Transformation to myelofibrosis occurred in 1 CMR patient, presumably because of the emergence of a different clone, raising questions about the relevance of CMR in ET patients. Grade 3 and 4 anemia occurred in 19% and 0% of ruxolitinib vs 0% (both grades) in the BAT arm, and grade 3 and 4 thrombocytopenia in 5.2% and 1.7% of ruxolitinib vs 0% (both grades) of BAT-treated patients. Rates of discontinuation or treatment switching did not differ between the 2 trial arms. The MAJIC-ET trial suggests that ruxolitinib is not superior to current second-line treatments for ET. This trial was registered at www.isrctn.com as #ISRCTN61925716. © 2017 by The American Society of Hematology.

Prolonged or Standard Infusion of Cefepime Hydrochloride in Treating Patients With Febrile Neutropenia

ClinicalTrials.gov

2017-05-25

Adult Acute Lymphoblastic Leukemia; Adult Acute Myeloid Leukemia; Adult Burkitt Lymphoma; Adult Diffuse Large Cell Lymphoma; Adult Diffuse Mixed Cell Lymphoma; Adult Diffuse Small Cleaved Cell Lymphoma; Adult Hodgkin Lymphoma; Adult Immunoblastic Large Cell Lymphoma; Adult Lymphoblastic Lymphoma; Atypical Chronic Myeloid Leukemia, BCR-ABL1 Negative; Breast Cancer; Chronic Eosinophilic Leukemia; Chronic Lymphocytic Leukemia; Chronic Myelogenous Leukemia; Chronic Myelomonocytic Leukemia; Chronic Neutrophilic Leukemia; Cutaneous T-cell Non-Hodgkin Lymphoma; Disseminated Neuroblastoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Grade 1 Follicular Lymphoma; Grade 2 Follicular Lymphoma; Grade 3 Follicular Lymphoma; Malignant Testicular Germ Cell Tumor; Mantle Cell Lymphoma; Marginal Zone Lymphoma; Multiple Myeloma; Mycosis Fungoides/Sezary Syndrome; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Neutropenia; Nodal Marginal Zone B-cell Lymphoma; Ovarian Epithelial Cancer; Ovarian Germ Cell Tumor; Plasma Cell Neoplasm; Poor Prognosis Metastatic Gestational Trophoblastic Tumor; Primary Myelofibrosis; Prolymphocytic Leukemia; Small Lymphocytic Lymphoma; Splenic Marginal Zone Lymphoma

A rare native mitral valve endocarditis successfully treated after surgical correction

PubMed Central

Garcia, Daniel C; Nascimento, Rhanderson; Soto, Victor; Mendoza, Cesar E

2014-01-01

Mycobacterium abscessus and Kocuria species are rare causes of infections in humans. Endocarditis by these agents has been reported in only 11 cases. M. abscessus is a particularly resistant organism and treatment requires the association of antibiotics for a prolonged period of time. We report a case of native mitral valve bacterial endocarditis due to M. abscessus and Kocuria species in a 48-year-old man with a history of intravenous drug use. The case was complicated by a perforation of the posterior mitral valve leaflet, leading to surgical mitral valve replacement. Cultures from the blood and mitral valve disclosed M. abscessus and Kocuria species. The patient was treated for 6 months with clarithromycin, imipenem and amikacin, with resolution of symptoms. Repeated blood cultures were negative. Acid-fast staining should be done in subacute endocarditis in order to identify rapidly growing mycobacteria. PMID:25270154

Predicting success in the treatment of psychopaths.

PubMed

Copas, J B; Whiteley, J S

1976-10-01

Factors from the social history of male psychopaths were examined in relation to their success or failure as measured by re-convictions of psychiatric hospital re-admissions 2-3 years after treatment at Henderson Hospital. From these data a weighted prediction formula was calculated and was tested out on a further cohort of patients. As a prediction instrument it was found to be reliable to a degree of significance just above the 1 per cent level. Other aspects of the subsequent career of treated patients are noted, in particular a tendency to early but often short-lived relapse and then longer standing success.

[Actinomyces empyema treated with decortications].

PubMed

Shteinberg, Michal; Perek, Shoshan; Ghanem, Nesrin; Sarafov, Israel; Peysakhovich, Yury; Adir, Yochai

2012-04-01

Actinomyces infections are rare infections, involving the head and neck, abdominal cavity, and the lung. We report a case of a 66 year old woman with shortness of breath and a pleural effusion from which Actinomyces meyeriwas cultured. The diagnosis was confirmed by the polymerase chain reaction technique. The infection was successfully treated with a combination of ampicillin and surgical decortication. Due to their rarity, Actinomyces infections are not often suspected. These infections are difficult to diagnose due to specific microbiologic requirements for isolation of Actinomyces. In many reviewed cases of Actinomyces infection, patients underwent surgery for presumed cancer but were eventually diagnosed as being infected with actinomycosis. Due to lack of improvement of our patient, surgical decortication was performed, which led to a successful outcome.

Hmga2 promotes the development of myelofibrosis in Jak2V617F knockin mice by enhancing TGF-β1 and Cxcl12 pathways.

PubMed

Dutta, Avik; Hutchison, Robert E; Mohi, Golam

2017-08-17

Myelofibrosis (MF) is a devastating blood disorder. The JAK2V617F mutation has been detected in ∼50% cases of MF. Elevated expression of high-mobility group AT hook 2 (HMGA2) has also been frequently observed in patients with MF. Interestingly, upregulation of HMGA2 expression has been found in association with the JAK2V617F mutation in significant cases of MF. However, the contribution of HMGA2 in the pathogenesis of MF remains elusive. To determine the effects of concurrent expression of HMGA2 and JAK2V617F mutation in hematopoiesis, we transduced bone marrow cells from Jak2 V617F knockin mice with lentivirus expressing Hmga2 and performed bone marrow transplantation. Expression of Hmga2 enhanced megakaryopoiesis, increased extramedullary hematopoiesis, and accelerated the development of MF in mice expressing Jak2 V617F Mechanistically, the data show that expression of Hmga2 enhances the activation of transforming growth factor-β1 (TGF-β1) and Cxcl12 pathways in mice expressing Jak2 V617F In addition, expression of Hmga2 causes upregulation of Fzd2, Ifi27l2a, and TGF-β receptor 2. Forced expression of Cxcl12, Fzd2, or Ifi27l2a increases megakaryocytic differentiation and proliferation in the bone marrow of Jak2 V617F mice, whereas TGF-β1 or Cxcl12 stimulation induces collagen deposition in the bone marrow mesenchymal stromal cells. Together, these findings demonstrate that expression of Hmga2 cooperates with Jak2 V617F in the pathogenesis of MF. © 2017 by The American Society of Hematology.

[Ultraminipercutaneous nephrolithotripsy in treating kidney stones].

PubMed

Martov, A G; Dutov, S V; Andronov, A S

2016-04-01

Percutaneous nephrolithotripsy (PNL) is the recommended method of surgical treatment of kidney stones of size greater than 2 cm. Trends in the development of modern urology have been steadily toward less traumatic method to treat nephrolithiasis - minimally invasive PNL. The present work aimed to explore of the possibilities of one of the modern variants of minimally invasive PNL - ultra-mini-PNL in treating nephrolithiasis. The study included 60 patients (mean age 45.6+/-7.2 years) with isolated kidney calculus, up to 2.0 cm or several stones with a total size of up to 2.5 cm. All patients were found to have 77 kidney stones, six of which had a size of 10 mm, 51 had a size of 11-15 mm and 20 had a size of 16-20 mm. 45% of patients had isolated renal pelvic stones and 28.3% had stones in the renal pelvis and lower calyx. All patients underwent ultra-mini-PNL using nephroscope size 7.5 Ch and tube size 12 Fr. The average duration of surgery from the moment of the puncture of the pyelocaliceal system to installing the nephrostomy tube was 65.4 minutes. Complete clearance of stones after single-stage ultra-mini-PNL was observed in 80% of cases. Nephrostomy tube was removed on days 2-3. The average postoperative hospital stay was 5.1 days. The most common complication was postoperative exacerbation of pyelonephritis (13.3% of patients), successfully treated with conservative measures. There were no cases of postoperative bleeding, accompanied by anemia and needed a blood transfusion. Considering high effectiveness and low rate of complications of ultra-mini-PNL, it can be successfully used in treating nephrolithiasis among a wide group of patients.

Color vision in an elderly patient with protanopic genotype and successfully treated unilateral age-related macular degeneration.

PubMed

Kitakawa, Takaaki; Hayashi, Takaaki; Tsuzuranuki, Satoshi; Kubo, Akiko; Tsuneoka, Hiroshi

2011-12-01

We investigated differences in color discrimination between the fellow eye and the affected eye successfully treated for unilateral age-related macular degeneration (AMD) in a 69-year-old male patient with protanopia. His best-corrected visual acuity (BCVA) was 1.2 in the right eye (RE) and 0.2 in the left eye (LE). Fundus and angiographic findings showed classic choroidal neovascularization (CNV) secondary to AMD in the LE. BCVA of the LE improved to 0.4, and CNV resolved by 15 months after initiating combined anti-vascular endothelial growth factor and photodynamic therapies. After CNV closure, the Farnsworth dichotomous was performed, showing confusion patterns of the protan axis in either eye. The Farnsworth-Munsell 100-hue test showed a total error score of 520 in the LE, much higher than the score of 348 in the RE. Complete genotypes of the long-wavelength-sensitive (L-) cone and middle-wavelength-sensitive (M-) cone opsin genes were determined by polymerase chain reaction, revealing that the patient had a single 5' L-M 3' hybrid gene (encoding an M-cone opsin), with this genotype responsible for protanopia (the L-cone opsin gene was non-functional), instead of the L-cone and M-cone opsin gene arrays. Poorer color vision discrimination in the LE than the RE remained present despite closure of CNV. The presence and type of congenital color vision defect can be confirmed using molecular genetic testing even if complications of acquired retinal diseases such as AMD are identified.

[A case of Stage IV gastric cancer was successfully treated with multi combination chemotherapy with S-1].

PubMed

Ami, Katsunori; Gokita, Kentarou; Kawai, Yousuke; Matsunaga, Yutarou; Fujiya, Keiichi; Ohshima, Nana; Amagasa, Hidetoshi; Ganno, Hideaki; Imai, Kenichirou; Fukuda, Akira; Nagahama, Takeshi; Ando, Masayuki; Okada, Youichi; Akita, Hidetaka; Tei, Shikofumi; Yamada, Yousuke; Takagi, Mariko; Kodaka, Fumi; Arai, Kuniyoshi

2014-11-01

Stage IV gastric cancer has poor prognosis, and median survival time (MST) is reported to range from 6 to 13 months. We report a case of long-term survival in a Stage IV gastric cancer patient who was successfully treated with multi combination chemotherapy with S-1. A 73-year-old woman presenting with gastric cancer with pyloric stenosis and peritoneal dissemination at the sigmoid colon underwent distal gastrectomy with D2 lymphadenectomy and sigmoidectomy. She received adjuvant chemotherapy with S-1 and CDDP after surgery. During the twelfth administration of S-1 and CDDP, she developed an anaphylactic reaction against CDDP; therefore, only S-1 was administered for the next 6 courses. Thirty one months postgastrectomy, a left ovarian metastasis (about 4 cm) was detected by computed tomography. Two courses of S-1 and CPT-11 were administered; however, the ovarian metastasis grew to twice its initial size. She underwent hysterectomy and bilateral ovariectomy. The pathological diagnosis was metastatic tumors in the uterus and ovary(Krukenberg tumor). After the second surgery, S-1 and docetaxel therapy was initiated. A metastasis (S2, 5mm diameter) appeared in the right lung around 65 months after the gastrectomy. The patient received a total of 28 courses, up until 69 months post-gastrectomy. At present, she hopes to finish the chemotherapy and is consulting a palliative care facility. At 80 months post-gastrectomy, she has no symptoms because the lung metastasis exhibits slow growth (15 mm diameter), and is maintaining her quality of life (QOL).

Successful Treatment of Human Plague with Oral Ciprofloxacin.

PubMed

Apangu, Titus; Griffith, Kevin; Abaru, Janet; Candini, Gordian; Apio, Harriet; Okoth, Felix; Okello, Robert; Kaggwa, John; Acayo, Sarah; Ezama, Geoffrey; Yockey, Brook; Sexton, Christopher; Schriefer, Martin; Mbidde, Edward Katongole; Mead, Paul

2017-03-01

The US Food and Drug Administration recently approved ciprofloxacin for treatment of plague (Yersina pestis infection) based on animal studies. Published evidence of efficacy in humans is sparse. We report 5 cases of culture-confirmed human plague treated successfully with oral ciprofloxacin, including 1 case of pneumonic plague.

Kleine-Levin Syndrome Treated With Clarithromycin

PubMed Central

Rezvanian, Elham; Watson, Nathaniel F.

2013-01-01

Kleine-Levin syndrome (KLS) is a rare sleep disorder characterized by periodic hypersomnia and various degrees of cognitive and behavioral disturbance, hyperphagia, and hypersexuality. Effective treatment is challenging. Stimulants marginally address sleepiness, but may increase irritability and do not improve cognitive and behavioral disturbances. Modafinil may shorten the symptomatic period but not the recurrence rate. Lithium and carbamazepine are beneficial in some cases, possibly related to similarities between KLS and affective disorders. Currently, no single medication is consistently successful in treating the syndrome. Here we report the short-term effect of clarithromycin in a patient with KLS. Citation: Rezvanian E; Watson NF. Kleine-Levin syndrome treated with clarithromycin. J Clin Sleep Med 2013;9(11):1211-1212. PMID:24235906

Increased risks of polycythemia vera, essential thrombocythemia, and myelofibrosis among 24 577 first-degree relatives of 11 039 patients with myeloproliferative neoplasms in Sweden

PubMed Central

Goldin, Lynn R.; Kristinsson, Sigurdur Y.; Helgadottir, Elin A.; Samuelsson, Jan; Björkholm, Magnus

2008-01-01

Previous small studies have reported familial clustering of myeloproliferative neoplasms (MPNs), including polycythemia vera (PV), essential thrombocythemia (ET), and myelofibrosis (MF). We identified 6217 PV, 2838 ET, 1172 MF, and 812 MPN unclassifiable (NOS) patients diagnosed in Sweden, 43 550 controls, and first-degree relatives of cases (n = 24 577) and controls (n = 99 542). Using a marginal survival model, we calculated relative risks (RRs) and 95% confidence intervals as measures of familial aggregation. Relatives of MPN patients had significantly increased risks of PV (RR = 5.7; 3.5-9.1), ET (RR = 7.4; 3.7-14.8), and MPN NOS (RR = 7.5; 2.7-20.8). Analyses stratified by type of first-degree relative revealed consistently higher risks for siblings, compatible with a model of recessive genetic inheritance, which can be confirmed only by identifying the susceptibility gene(s). Mean age at MPN diagnosis was not different (P = .20) for affected relatives of cases (57.5 years) versus controls (60.6 years), and risk of MPN by age was not different for parents versus offspring of MPN cases (P = .10), providing no support for anticipation. Relatives of MPN patients had a borderline increased risk of chronic myeloid leukemia (CML; RR = 1.9; 0.9-3.8; P = .09). Our findings of 5- to 7-fold elevated risk of MPNs among first-degree relatives of MPN patients support the hypothesis that common, strong, shared susceptibility genes predispose to PV, ET, MF, and possibly CML. PMID:18451307

The prognosis of root canal therapy: a 10-year retrospective cohort study on 411 patients with 1175 endodontically treated teeth.

PubMed

Fonzar, Federica; Fonzar, Alberto; Buttolo, Piercarlo; Worthington, Helen V; Esposito, Marco

2009-01-01

To evaluate the 10-year prognosis of consecutively endodontically treated or retreated teeth and to investigate some of the prognostic factors which could predict the long-term outcome of endodontic therapy. This retrospective cohort study included any patient who had endodontically treated or retreated teeth from 1986 to 1998 by a single operator in a private practice. Outcome measures were clinical and radiographic success assessed by the operator, radiographic success assessed by an independent outcome assessor and complications evaluated 10 years after treatment. Descriptive statistics, life table, Kaplan-Meier and Cox regression analyses for success were fitted. A total of 411 patients with 1175 endodontically treated teeth were identified. Ten years after treatment 102 patients (24.8%) with 223 (19.0%) teeth were lost at the follow-up. The number of teeth that were originally treated and retreated were 704 and 471, respectively. Thirty-two teeth (2.7%) had one complication, which was successfully treated. A total of 988 (84.1%) teeth were considered a complete success, 46 (3.9%) a partial success, 52 (4.4%) a partial failure and 68 (5.8%) had to be extracted according to the treating clinician. For 21 teeth (1.8%) there was no follow-up information. The radiographic healing of 1086 teeth was evaluated by an independent assessor: 980 (90.2%) showed complete healing, 52 (4.8%) improvement, and 54 (5.0%) no change or worsening. The life-table analysis showed 93% of teeth surviving at 10 years after endodontic treatment. There were no differences for survival rates between teeth treated for the first time and those that were retreated (Kaplan-Meier). Teeth retreated because of symptoms or for a periapical/lateral radiolucency were more likely to fail. Approximately 7% of endodontically treated teeth were extracted 10 years after treatment. Symptoms and radiolucency of teeth needing retreatment may be important predictors for failure.

Pulsed Radiofrequency Ablation for Treating Sural Neuralgia.

PubMed

Abd-Elsayed, Alaa; Jackson, Markus; Plovanich, Elizabeth

2018-01-01

Sural neuralgia is persistent pain in the distribution of the sural nerve that provides sensation to the lateral posterior corner of the leg, lateral foot, and fifth toe. Sural neuralgia is a rare condition but can be challenging to treat and can cause significant limitation. We present 2 cases of sural neuralgia resistant to conservative management that were effectively treated by pulsed radiofrequency ablation. A 65-year-old female developed sural neuralgia after a foot surgery and failed conservative management. She had successful sural nerve blocks, and pulsed radiofrequency ablation led to an 80% improvement in her pain. A 33-year-old female presented with sural neuralgia secondary to two falls. The patient had tried several conservative modalities with no success. We performed diagnostic blocks and pulsed radiofrequency ablation, and the patient reported 80% improvement in her pain. Pulsed radiofrequency ablation may be a safe and effective treatment for patients with sural neuralgia that does not respond to conservative therapy. However, studies are needed to elucidate its effectiveness and safety profile.

Successful Treatment of Human Plague with Oral Ciprofloxacin

PubMed Central

Apangu, Titus; Griffith, Kevin; Abaru, Janet; Candini, Gordian; Apio, Harriet; Okoth, Felix; Okello, Robert; Kaggwa, John; Acayo, Sarah; Ezama, Geoffrey; Yockey, Brook; Sexton, Christopher; Schriefer, Martin; Mbidde, Edward Katongole

2017-01-01

The US Food and Drug Administration recently approved ciprofloxacin for treatment of plague (Yersina pestis infection) based on animal studies. Published evidence of efficacy in humans is sparse. We report 5 cases of culture-confirmed human plague treated successfully with oral ciprofloxacin, including 1 case of pneumonic plague. PMID:28125398

Successful psychotherapy for psychogenic seizures in men.

PubMed

Quinn, Maria Clare; Schofield, Margot J; Middleton, Warwick

2012-01-01

This qualitative study examined factors contributing to the development and successful treatment of psychogenic non-epileptic seizures (PNES), in civilian men. In-depth interviews were conducted with therapist-client dyads comprising two male clients who had been successfully treated for PNES and their therapists. A theory-building case study approach provided evidence that those factors known to contribute to PNES and other somatoform symptoms in females and in males engaged in war also contributed to these symptoms in these two civilian males. In addition, PNES in these civilian males occurred in contexts where masculine identity was developmentally curtailed and socially constrained. Successful treatments occurred in long-term therapeutic relationships that sanctioned verbal expression of strong emotion and provided the attunement necessary for development of a robust masculine identity. These findings have implications for the funding of therapy, and training of therapists.

Fludarabine Phosphate, Cyclophosphamide, Total Body Irradiation, and Donor Stem Cell Transplant in Treating Patients With Blood Cancer

ClinicalTrials.gov

2018-06-13

Accelerated Phase Chronic Myelogenous Leukemia, BCR-ABL1 Positive; Acute Leukemia in Remission; Acute Lymphoblastic Leukemia; Acute Myeloid Leukemia; Acute Myeloid Leukemia With FLT3/ITD Mutation; Acute Myeloid Leukemia With Gene Mutations; Aplastic Anemia; B-Cell Non-Hodgkin Lymphoma; CD40 Ligand Deficiency; Chronic Granulomatous Disease; Chronic Leukemia in Remission; Chronic Lymphocytic Leukemia; Chronic Myelogenous Leukemia, BCR-ABL1 Positive; Chronic Myelomonocytic Leukemia; Chronic Phase Chronic Myelogenous Leukemia, BCR-ABL1 Positive; Congenital Amegakaryocytic Thrombocytopenia; Congenital Neutropenia; Congenital Pure Red Cell Aplasia; Glanzmann Thrombasthenia; Immunodeficiency Syndrome; Myelodysplastic Syndrome; Myelofibrosis; Myeloproliferative Neoplasm; Paroxysmal Nocturnal Hemoglobinuria; Plasma Cell Myeloma; Polycythemia Vera; Recurrent Non-Hodgkin Lymphoma; Refractory Non-Hodgkin Lymphoma; Secondary Acute Myeloid Leukemia; Secondary Myelodysplastic Syndrome; Severe Aplastic Anemia; Shwachman-Diamond Syndrome; Sickle Cell Disease; T-Cell Non-Hodgkin Lymphoma; Thalassemia; Waldenstrom Macroglobulinemia; Wiskott-Aldrich Syndrome

The current state of GPCR-based drug discovery to treat metabolic disease.

PubMed

Sloop, Kyle W; Emmerson, Paul J; Statnick, Michael A; Willard, Francis S

2018-02-02

One approach of modern drug discovery is to identify agents that enhance or diminish signal transduction cascades in various cell types and tissues by modulating the activity of GPCRs. This strategy has resulted in the development of new medicines to treat many conditions, including cardiovascular disease, psychiatric disorders, HIV/AIDS, certain forms of cancer and Type 2 diabetes mellitus (T2DM). These successes justify further pursuit of GPCRs as disease targets and provide key learning that should help guide identifying future therapeutic agents. This report reviews the current landscape of GPCR drug discovery with emphasis on efforts aimed at developing new molecules for treating T2DM and obesity. We analyse historical efforts to generate GPCR-based drugs to treat metabolic disease in terms of causal factors leading to success and failure in this endeavour. © 2018 The British Pharmacological Society.

Hereditary angioneurotic edema treated by partial uvulectomy.

PubMed

Waeckerle, J F; Smith, H A; McNabney, W K

1976-06-01

Hereditary angioneurotic edema (HANE) is a rare familial disease of C1 esterase inhibitor deficiency that produces recurring attacks of acute, circumscribed, noninflammatory edema. The technique of partial uvulectomy to treat HANE can reduce the mortality from this condition due to asphyxiation. Three cases in which partial uvulectomy was the successful mode of treatment are described.

A case of chemotherapy-induced congestive heart failure successfully treated with Chinese herbal medicine.

PubMed

Wu, Bei-Yu; Liu, Chun-Ting; Chen, Shih-Yu; Tsai, Ming-Yen

2015-04-01

A case is presented to illustrate a potential effect of Chinese herbal medicine (CHM) formulas in treating chemotherapy-induced cardiotoxicity. An 18-year-old adolescent male with refractory acute lymphoblastic leukemia (ALL) had experienced anthracycline-induced congestive heart failure (CHF) for 3 weeks. Under intensive care with conventional therapy, the patient still had exercise intolerance and depended on supplemental oxygen all day. Therefore, he consented to treatment with traditional Chinese medicine (TCM) for alternative therapy. This patient was treated with modified Zhi Gan Cao Tang (ZGCT), three times a day for 2 months. After 6 days of CHM treatment, the patient could tolerate daily activity without supplemental oxygen. After 2 months of CHM treatment, the follow-up chest X-ray showed great improvements in pulmonary edema and cardiomegaly. In this case, anthracycline-induced cardiotoxicity resolved slowly following the administration of modified ZGCT. It is suggested that the CHM formula has a protective effect on the progression of CHF secondary to the use of anthracyclines in pediatric cancer. Further studies to determine the mechanism and clinical trials are warranted. Copyright © 2015 Elsevier Ltd. All rights reserved.

Severe gastric variceal bleeding successfully treated by emergency splenic artery embolization.

PubMed

Sankararaman, Senthilkumar; Velayuthan, Sujithra; Vea, Romulo; Herbst, John

2013-06-01

Bleeding from gastric varices due to splenic vein obstruction is extremely rare in children, but it can be catastrophic. Reported herein is the case of a teenager with splenic vein thrombosis and chronic decompensated liver disease from autoimmune hepatitis who presented with massive gastric variceal bleeding. Standard medical management did not control the bleeding. Due to decompensated liver disease and continuous active bleeding, emergency partial splenic artery embolization was preferred over splenectomy or a shunt procedure. Bleeding was successfully controlled by partial splenic artery embolization by decreasing the inflow of blood into the portal system. It is concluded that emergency partial splenic artery embolization is a safer alternative life-saving procedure to manage severe gastric variceal bleeding due to splenic vein obstruction in a patient with high surgical risk. To our knowledge, only one other patient with similar management has been reported in the pediatric age group. © 2013 The Authors. Pediatrics International © 2013 Japan Pediatric Society.

EXEL-8232, a small-molecule JAK2 inhibitor, effectively treats thrombocytosis and extramedullary hematopoiesis in a murine model of myeloproliferative neoplasm induced by MPLW515L.

PubMed

Wernig, G; Kharas, M G; Mullally, A; Leeman, D S; Okabe, R; George, T; Clary, D O; Gilliland, D G

2012-04-01

About 10% of patients with essential thrombocythemia (ET) or myelofibrosis (MF) that lack mutations in JAK2 harbor an activating mutation in the thrombopoietin receptor, MPLW515L. Distinct from the JAK2V617F retroviral transplant model, the MPLW515L model recapitulates many features of ET and MF, including severe fibrosis and thrombocytosis. We have tested EXEL-8232, an experimental potent JAK2 inhibitor, for efficacy in suppression of thrombocytosis in vivo and for its ability to attenuate MPLW515L myeloproliferative disease. EXEL-8232 was administered for 28 days q12 h by oral gavage at doses of 30 or 100 mg/kg, prospectively. Animals treated with EXEL-8232 at 100 mg/kg had normalized high platelet counts, eliminated extramedullary hematopoiesis in the spleen and eliminated bone marrow fibrosis, whereas the wild-type controls did not develop thrombocytopenia. Consistent with a clinical response in this model, we validated surrogate end points for response to treatment, including a reduction of endogenous colony growth and signaling inhibition in immature erythroid and myeloid primary cells both in vitro and upon treatment in vivo. We conclude that EXEL-8232 has efficacy in treatment of thrombocytosis in vivo in a murine model of ET and MF, and may be of therapeutic benefit for patients with MPL-mutant MPN.

Epilepsy characteristics and psychosocial factors associated with ketogenic diet success.

PubMed

McNamara, Nancy A; Carbone, Loretta A; Shellhaas, Renée A

2013-10-01

The ketogenic diet is an effective therapy for childhood epilepsy, but its important impacts on families could affect successful treatment. We assessed medical and psychosocial factors associated with successful ketogenic diet treatment. A total of 23 families of patients treated with ketogenic diet completed questionnaires (30% response), including inquiries about challenges to successful dietary treatments and validated family functioning scales. Of these, 14 were considered successful (diet discontinued once the child was seizure-free or continued as clinically indicated). Family-identified challenges were food preparation time (n = 11) and that the diet was too restrictive (n = 9). Neither Medicaid insurance nor family functioning scale scores were significantly associated with successful treatment. Lower seizure frequency prior to ketogenic diet initiation (P = .02) and postdiet seizure improvement (P = .01) were associated with increased odds of success. Effective ketogenic diet treatment is dictated both by psychosocial and epilepsy-related influences. A focus on understanding the psychosocial issues may help to improve families' experiences and success with the ketogenic diet.

Moxidectin toxicosis in a puppy successfully treated with intravenous lipids.

PubMed

Crandell, Dawn E; Weinberg, Guy L

2009-04-01

To describe successful treatment of canine moxidectin toxicosis with the novel therapy of IV lipid administration. A 16-week-old female Jack Russell Terrier was presented with acute onset of seizures followed by paralysis and coma shortly following suspected exposure to an equine formulation of moxidectin. Moxidectin toxicity was later confirmed. Initial therapy consisted of diazepam, glycopyrrolate, and IV fluids. Mechanical ventilation and supportive nursing care were provided as needed. An emulsion of 20% soybean oil in water, commonly used as the fat component of parenteral nutrition, was administered intravenously as a bolus of 2 mL/kg followed by 4 mL/kg/h for 4 hours beginning 10 hours after exposure and was administered again at a rate of 0.5 mL/kg/min for 30 minutes beginning 25.5 hours post-exposure. Mild improvement was seen after the first dose, and dramatic improvement was noted within 30 minutes of the second dose. The puppy's neurologic status returned to normal within 6 hours of the second administration, with no relapses. IV lipid therapy is a novel treatment approach for moxidectin toxicity. Its use is supported by recent research and case studies involving IV lipid administration for bupivacaine and other fat-soluble toxins. Lipid administration appeared to reverse the signs of toxicity and may prove to be a highly effective therapy for moxidectin and other fat-soluble toxins.

Willingness to treat drug dependence and depression: comparisons of future health professionals

PubMed Central

Ahmedani, Brian K; Kubiak, Sheryl Pimlott; Rios-Bedoya, Carlos F; Mickus, Maureen; Anthony, James C

2011-01-01

Purpose Stigma-related feelings, including degree of enthusiasm and willingness to work with alcohol, drug, and mental disorder (ADM) patients, as well as anticipated success in such work, will be required for the United States to be successful in its new initiatives for ADM screening, brief intervention, and effective referral to treatment and rehabilitation services (SBIRT). This study investigates students of medicine and social work with respect to their stigma-related feelings and degree of enthusiasm or willingness to treat patients affected by alcohol dependence, nicotine dependence, or major depression. Inference is strengthened by an anonymous online survey approach, with use of randomized reinforcers to gain at least partial experimental control of nonparticipation biases that otherwise are present in student survey data. Material and methods All students on required course rosters were asked to participate in a two-part in-class and online assessment; 222 participated, with a gradient of participation induced via randomly drawn reinforcers for online survey participation. Between-group comparisons were made with a multivariate generalized linear model and generalized estimating equations approach that adjusts for covariates. Results Medical and social work students did not differ from each other with respect to their willingness to treat patients affected by major depression, alcohol dependence, or nicotine dependence, but together were less willing to treat nicotine and alcohol dependence-affected patients as compared to depression-affected patients. Personal history was not associated with the students’ willingness to treat, but men were less willing to treat. Drawing strength from the randomized reinforcer experimental design nested within this survey approach, the study evidence suggests potential nonparticipation bias in standard surveys on this topic. Conclusion These results indicate that future health professionals may prefer to treat

Nonsmall Cell Lung Carcinoma with Giant Cell Features Expressing Programmed Death-Ligand 1: A Report of a Patient Successfully Treated with Pembrolizumab

PubMed Central

Nakayama, Shingo; Sasaki, Mamoru; Morinaga, Shojiroh

2018-01-01

Giant cell carcinoma, a rare variant of nonsmall cell lung carcinoma (NSCLC), is characterized by aggressive progression and poor response to conventional chemotherapy. This report is the first to describe a patient with NSCLC and giant cell features who was successfully treated with pembrolizumab, an antibody targeting programmed death-1 (PD-1). A 69-year-old woman was diagnosed with NSCLC with multiple brain metastases. Histological evaluation of lung biopsy specimens revealed proliferation of pleomorphic giant tumor cells with poor cohesiveness, findings consistent with giant cell carcinoma. Immunostaining showed that a high proportion of the tumor cells were positive for expression of programmed death-ligand 1 (PD-L1). The patient received stereotactic radiotherapy for the brain metastases, followed by administration of pembrolizumab. Treatment with pembrolizumab resulted in the rapid regression of the primary lung nodule, with the progression-free period maintained for at least four treatment cycles. Immunotherapy targeting PD-1/PD-L1 may be an option for patients with PD-L1-positive NSCLC with giant cell features. PMID:29736285

Nonsmall Cell Lung Carcinoma with Giant Cell Features Expressing Programmed Death-Ligand 1: A Report of a Patient Successfully Treated with Pembrolizumab.

PubMed

Nakayama, Shingo; Sasaki, Mamoru; Morinaga, Shojiroh; Minematsu, Naoto

2018-01-01

Giant cell carcinoma, a rare variant of nonsmall cell lung carcinoma (NSCLC), is characterized by aggressive progression and poor response to conventional chemotherapy. This report is the first to describe a patient with NSCLC and giant cell features who was successfully treated with pembrolizumab, an antibody targeting programmed death-1 (PD-1). A 69-year-old woman was diagnosed with NSCLC with multiple brain metastases. Histological evaluation of lung biopsy specimens revealed proliferation of pleomorphic giant tumor cells with poor cohesiveness, findings consistent with giant cell carcinoma. Immunostaining showed that a high proportion of the tumor cells were positive for expression of programmed death-ligand 1 (PD-L1). The patient received stereotactic radiotherapy for the brain metastases, followed by administration of pembrolizumab. Treatment with pembrolizumab resulted in the rapid regression of the primary lung nodule, with the progression-free period maintained for at least four treatment cycles. Immunotherapy targeting PD-1/PD-L1 may be an option for patients with PD-L1-positive NSCLC with giant cell features.

Glycopyrrolate-induced craniofacial compensatory hyperhidrosis successfully treated with oxybutynin: report of a novel adverse effect and subsequent successful treatment.

PubMed

Prouty, Megan E; Fischer, Ryan; Liu, Deede

2016-10-15

Hyperhidrosis, or abnormally increased sweating, is a condition that may have a primary or secondary cause. Usually medication- induced secondary hyperhidrosis manifests with generalized, rather than focal sweating. We report a 32-year-old woman with a history of palmoplantar hyperhidrosis for 15 years who presented for treatment and was prescribed oral glycopyrrolate. One month later, the palmoplantar hyperhidrosis had resolved, but she developed new persistent craniofacial sweating. After an unsuccessful trial of clonidine, oxybutynin resolved the craniofacial hyperhidrosis. To our knowledge, this is the first case of compensatory hyperhidrosis secondary to glycopyrrolate reported in the literature. The case highlights the importance of reviewing medication changes that correlate with new onset or changing hyperhidrosis. It also demonstrates a rare drug adverse effect with successful treatment.

Treat to target in gout.

PubMed

Perez-Ruiz, Fernando; Moreno-Lledó, Aitana; Urionagüena, Irati; Dickson, Alastair J

2018-01-01

The treat-to-target (T2T) approach has been successfully implemented in a number of diseases. T2T has been proposed for rheumatic diseases such as RA, spondyloarthritis, lupus, and recently for gout. The level of evidence for such approaches differs from one condition to the other (moderate to high for hyperlipidaemia, for example). Practice is based on the best available evidence at any time, and in absence of good evidence for T2T in gout, some suggest a conservative only-treat-symptoms approach. Evidence suggests that not treating gout to target in the long term is overall associated with worsening outcomes, such as flares, tophi and structural damage, which is associated to loss of quality of life and mortality. Different targets have been proposed for hyperuricaemia in gout; lower than 6 mg/dl (0.36 mmol/l) for all patients, at least <5 mg/dl (0.30 mmol/l) for patients with severe-polyarticular or tophaceous-gout. © The Author 2018. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

A case of pulmonary carcinoid tumour in a pregnant woman successfully treated with bronchoscopic (electrocautery) therapy

PubMed Central

Binesh, Fariba; Samet, Mohammad; Bovanlu, Taghi Roshan

2013-01-01

We present an uncommon case of a carcinoid tumour of the bronchus that was diagnosed during pregnancy in a 28-year-old woman. The patient was admitted at the emergency department with massive haemoptysis. Owing to the patient's critical condition, she underwent urgent flexible bronchoscopy. Bleeding was controlled by local injection of 500 mg tranexamic acid and electrocautery. After the bleeding has stopped, multiple specimens were taken. Histological examination confirmed typical carcinoid tumour. Owing to repeated haemoptysis, she was treated with bronchoscopic (electrocautery) therapy, and, after delivery, she underwent pulmonary lobectomy. Only a few similar cases were found in the literature reporting bronchopulmonary carcinoid tumour during pregnancy and we could not find any similar case which was treated by electrocautery. PMID:23608865

The success of primary chemotherapy for group D heritable retinoblastoma.

PubMed

Cohen, V M L; Kingston, J; Hungerford, J L

2009-07-01

To report the ocular survival and event-free survival following primary multiagent chemotherapy for group D, heritable bilateral retinoblastoma (RB). The RB database was used to identify children with heritable, bilateral RB treated with primary chemotherapy (six cycles of vincristine, etoposide and carboplatin). Only Group D eyes with more than 12 months' follow-up were analysed. The timing, number and type of salvage treatments were recorded. Kaplan-Meier estimates for the ocular survival and event-free survival (percentage of eyes that avoided external beam radiotherapy and/or enucleation) were performed as a function of time. Of 18 group D eyes, two (11%) were treated successfully with chemotherapy alone, nine (50%) underwent successful salvage treatment, and seven (39%) were enucleated. The median time from completing chemotherapy to enucleation was 9 months (range 4 to 25 months). Ocular survival was 67% at 2 years. External beam radiotherapy proved successful salvage treatment in five of nine eyes, so the event-free survival was 34% at 2 years. Multiagent chemotherapy alone is rarely sufficient for the preservation of group D eyes. External beam radiotherapy and plaque radiotherapy remain important salvage treatments for advanced, heritable retinoblastoma.

Sleep bruxism possibly triggered by multiple sclerosis attacks and treated successfully with botulinum toxin: Report of three cases.

PubMed

Sevim, Serhan; Kaleağası, Hakan; Fidancı, Halit

2015-09-01

Sleep bruxism refers to a nocturnal parafunctional activity including the clenching, grinding or gnashing of teeth. While most of the nocturnal bruxism cases seen in the general population are apparently idiopathic, it has been reported to be associated with a range of neurological diseases such as Huntington's disease, cranio-cervical dystonia and post-anoxic brain damage, but not multiple sclerosis (MS). We describe three cases of MS patients who have had moderate to severe complaints of bruxism in the two weeks following their relevant MS attacks. None of the three patients had a diagnosis of bruxism prior to her attack. The diagnosis was confirmed in one out of three by a polysomnography. One patient did not have any complaints related to bruxism previous to her attack, whereas two had mild and infrequent complaints. The symptoms of the relevant attacks were left hemihypesthesia in all and hemiparesis in two. None of the patients had spasticity that could result in severe teeth clenching. All three patients presented with morning headaches and jaw pain or tightness and were treated successfully with botulinum toxin (Btx) injections applied to their masseter and temporalis muscles. The cause of bruxism is controversial but lesions of the cortico-basalganglia-thalamo-cotrical loops are thought to be most likely. However, acute or chronic lesions in those pathways were not demonstrated in the 3 patients. It is feasible that they had normal appearing white matter interruptions in their cortico-basalganglia-thalamocortical loops along with their relevant attack. Copyright © 2015 Elsevier B.V. All rights reserved.

Rare congenital chromosomal aberration dic(X;Y)(p22.33;p11.32) in a patient with primary myelofibrosis.

PubMed

Pavlistova, Lenka; Izakova, Silvia; Zemanova, Zuzana; Bartuskova, Lucie; Langova, Martina; Malikova, Pavlina; Michalova, Kyra

2016-01-01

Constitutional translocations between sex chromosomes are rather rare in humans with breakpoints at Xp11 and Yq11 as the most frequent. Breakpoints on the short arm of the Y chromosome form one subgroup of t(X;Y), giving rise to a derived chromosome with the centromeres of both the X and Y chromosomes, dic(X;Y). Here, we report a rare congenital chromosomal aberration, 46,X,dic(X;Y)(p22.33;p11.32)[20]/45,X[10], in an adult male. Primary myelofibrosis, a malignant haematological disease, was diagnosed in a 63-year-old man following liver transplantation after hepatocellular carcinoma. By the analysis of the bone marrow sample, the karyotype 46,X,dic(X;Y)(p22.33;p11.32) was detected in all the mitoses analysed and verified with multicolour fluorescence in situ hybridization (mFISH). A cytogenetic examination of stimulated peripheral blood cells revealed the constitutional karyotype 46,X,dic(X;Y)(p22.33;p11.32)[20]/45,X[10]. The cell line 45,X was confirmed with FISH in 35 % of interphase nuclei. The SRY locus was present on the dicentric chromosome. A CGH/SNP array (Illumina) revealed a gain of 153,7 Mbp of the X chromosome and a 803-kbp microdeletion (including the SHOX gene), which were also confirmed with FISH. SHOX encodes a transcriptional factor that regulates the growth of the long bones. The deletion of the SHOX gene together with the Madelung deformity of the forearm and the short stature of the proband led to a diagnosis of Léri-Weill dyschondrosteosis (LWD). The gain of almost the whole X chromosome (153,7 Mbp) was considered a variant of Klinefelter syndrome (KS). The levels of gonadotropins and testosterone were consistent with gonadal dysfunction. A malformation of the right external ear was detected. We have reported a structural aberration of the sex chromosomes, dic(X;Y)(p22.33;p11.32). The related genomic imbalance is associated with two known hereditary syndromes, LWD and a KS variant, identified in our proband at an advanced age. Because the

[Gastric stromal tumor treated by laparoscopic surgery].

PubMed

Alecu, L; Costan, I; Vitalariu, A; Obrocea, F; Păcuraru, Elena; Gulinescu, L

2002-01-01

The authors describe a 59 years old female patient, with a gastrointestinal stromal tumor located on the posterior wall of the gastric funds, who was treated successfully by laparoscopic wedge resection (with clear resection margins), through an anteriorly placed gastrotomy, thus allowing an endoscopic linear cutter Endo GIA, to excise the tumor with a cuff of normal gastric tissue. The anterior gastrotomy was performed with Ultra-Shears. Delivery of the tumor through the gastrotomy is essential for success. The operative time was 110 minutes. The tumor was diagnosed as a gastrointestinal stromal submucosal tumor (of low-grade malignancy) and immunohistochemicaly, this tumor was positive for CD 34. Posterior gastric tumor can be removed using laparoscopic surgery.

A case of recurrent psychosis during sickle cell disease crisis treated successfully with ziprasidone.

PubMed

Spiegel, David R; Messerschmidt, Cory; Morewitz, Jerry; Akintola, Morakinyo

2013-01-01

We present and follow a series of three consecutive hospitalizations of a 36-year-old, African-American male with sickle cell anemia disease who presented with sickle cell crises and a new onset psychotic episode. After multiple hospitalizations for prior episodes of sickle cell crisis-induced pain, treated with rehydration, blood transfusions, and opiate medication, this hospitalization was the first time he developed psychosis. As such, we discuss the differential diagnosis of the latter, and effective adjunctive treatment with ziprasidone.

Case Report: Diagnosis of a Rare Plaque-Like Dermal Fibroma Successfully Treated With Mohs Surgery.

PubMed

Gill, Pavandeep; Arlette, John; Shiau, Carolyn J; Abi Daoud, Marie S

CD34-positive plaque-like dermal fibroma (PDF) is a poorly characterised benign dermal neoplasm that has a wide differential diagnosis. It can be mistaken for other entities on superficial biopsy and be overtreated, leading to unnecessary worry and extensive surgery. To report on an uncommon presentation of this entity, the histopathologic differential diagnosis of PDF, and a novel treatment method. Clinical and histopathological information was obtained for a PDF lesion on a 75-year-old man. On superficial biopsy, the PDF lesion was misinterpreted as a possible neurothekeoma. Successful Mohs surgery and genetic testing confirmed the diagnosis of PDF, and the patient received appropriate tissue-sparing surgical management. This case adds to our current knowledge about PDF and highlights the importance of early recognition of these lesions to direct appropriate diagnostic testing (full-thickness biopsy) and management. This case confirms successful management with Mohs surgery.

Successful use of camelid (alpaca) antivenom to treat a potentially lethal tiger snake (Notechis scutatus) envenomation in a dog.

PubMed

Padula, Andrew M; Winkel, Kenneth D

2016-05-01

This report describes a confirmed clinical case of tiger snake (Notechis scutatus) envenomation in a domestic dog that was successfully treated with a novel polyvalent camelid (alpaca; Llama pacos) antivenom. Samples collected from the dog were assayed for tiger snake venom (TSV) using a highly sensitive and specific ELISA. The TSV concentration in serum and urine at initial presentation was 365 ng/mL and 11,640 ng/mL respectively. At the time of initial presentation whole blood collected from the dog did not clot and the Prothrombin Time was abnormally increased (>300 s). Serum was also visibly hemolysed. The dog was administered antihistamine, dexamethasone and 4000 Units (sufficient to neutralise 40 mg of TSV) of a novel polyvalent alpaca antivenom diluted in 0.9% NaCl. At 4 h post-antivenom treatment the dog's clinical condition had improved markedly with serum TSV concentrations below the limit of detection (<0.015 ng/mL), consistent with complete binding of venom antigens by the alpaca antivenom. Coagulation parameters had begun to improve by 4 h and had fully normalised by 16 h post-antivenom. Venom concentrations in both serum and urine remained undetectable at 16 h post-antivenom. The dog made a complete recovery, without complications, suggesting that the alpaca-based antivenom is both clinically safe and effective. Copyright © 2016 Elsevier Ltd. All rights reserved.

Successful Surgical Treatment for Elephantiasis Nostras Verrucosa Using a New Designed Column Flap.

PubMed

Han, Hyun Ho; Lim, Soo Yeon; Oh, Deuk Young

2015-09-01

Elephantiasis nostras verrucosa is a chronic lymphedema that causes enlarged and disfigured extremities. There are plenty of treatment options. However, there is no complete treatment. Preventive or symptomatic therapy is the basis for treating elephantiasis. In this article, we report a case of elephantiasis nostras verrucosa treated successfully by surgical reconstruction using a newly designed column flap. © The Author(s) 2015.

Ruptured Aortic Aneurysm From Late Type II Endoleak Treated by Transarterial Embolization

DOE Office of Scientific and Technical Information (OSTI.GOV)

Gunasekaran, Senthil, E-mail: sgunasekaran@lumc.edu; Funaki, Brian, E-mail: bfunaki@radiology.bsd.uchicago.edu; Lorenz, Jonathan, E-mail: jlorenz@radiology.bsd.uchicago.edu

2013-02-15

Endoleak is the most common complication after endovascular aneurysm repair. The most common type of endoleak, a type II endoleak, typically follows a benign course and is only treated when associated with increasing aneurysm size. In this case report, we describe a ruptured abdominal aortic aneurysm due to a late, type II endoleak occurring 10 years after endovascular aneurysm repair that was successfully treated by transarterial embolization.

Fluoroscopically guided large balloon dilatation for treating congenital esophageal stenosis in children.

PubMed

Hu, Hong-Tao; Shin, Ji Hoon; Kim, Jin-Hyoung; Jang, Jong Keon; Park, Jung-Hoon; Kim, Tae-Hyung; Nam, Deok Ho; Song, Ho-Young

2015-07-01

We aimed to evaluate the safety and clinical effectiveness of fluoroscopically guided large balloon dilatation for treating congenital esophageal stenosis in children. Our study included seven children (mean age 4.0 years) who underwent a total of ten balloon dilatation sessions. The initial balloon diameters were 10-15 mm. The technical success, clinical success (improved food intake and reduced dysphagia within 1 month following the first balloon dilatation), dysphagia recurrence, and complications were retrospectively evaluated. Technical and clinical success rates were 100 %. During the mean 38-month follow-up period after the first balloon dilatation, 3 (43 %) patients underwent only one additional balloon dilatation 4-5 months after the first balloon dilatation for dysphagia recurrence. Two of them showed improvement without further recurrence, while the remaining one underwent partial esophagectomy. Well-contained transmural esophageal rupture (type 2) occurred in two (29 %, 2/7) patients and during two (20 %, 2/10) balloon dilatation sessions. All ruptures were successfully treated conservatively. Our study showed that fluoroscopically guided large balloon dilatation seems to be a simple and effective primary treatment technique for congenital esophageal stenosis in children. Esophageal ruptures were not uncommon although they were not fatal.

The Successful Treatment of Elephantiasis Nostras Verrucosa With Ablative Carbon Dioxide Laser.

PubMed

Robinson, Caitlin G; Lee, Kory R; Thomas, Valencia D

2018-03-01

Elephantiasis nostras verrucosa (ENV) is a disfiguring skin condition that is difficult to treat. Existing treatment modalities serve to improve cosmesis or treat symptoms. Herein, we report a case of ENV with lymphocutaneous fistula successfully treated with ablative carbon dioxide laser. A 57-year-old woman with biopsy-proven ENV with lymphocutaneous fistula was treated with ablative carbon dioxide laser to the symptomatic area of her right thigh in 3 treatment sessions over 6 months. The patient had resolution of lymphocutaneous drainage as well as 90% improvement in the appearance of ENV lesions at the 1-month follow-up visit. Ablative carbon dioxide laser may provide cosmetic, symptomatic, and medical benefit for patients with localized ENV.

Recurrent Scedosporium apiospermum mycetoma successfully treated by surgical excision and terbinafine treatment: a case report and review of the literature.

PubMed

Tóth, Eszter J; Nagy, Géza R; Homa, Mónika; Ábrók, Marianna; Kiss, Ildikó É; Nagy, Gábor; Bata-Csörgő, Zsuzsanna; Kemény, Lajos; Urbán, Edit; Vágvölgyi, Csaba; Papp, Tamás

2017-04-14

Scedosporium apiospermum is an emerging opportunistic filamentous fungus, which is notorious for its high levels of antifungal-resistance. It is able to cause localized cutaneous or subcutaneous infections in both immunocompromised and immunocompetent persons, pulmonary infections in patients with predisposing pulmonary diseases and invasive mycoses in immunocompromised patients. Subcutaneous infections caused by this fungus frequently show chronic mycetomatous manifestation. We report the case of a 70-year-old immunocompromised man, who developed a fungal mycetomatous infection on his right leg. There was no history of trauma; the aetiological agent was identified by microscopic examination and ITS sequencing. This is the second reported case of S. apiospermum subcutaneous infections in Hungary, which was successfully treated by surgical excision and terbinafine treatment. After 7 months, the patient remained asymptomatic. Considering the antifungal susceptibility and increasing incidence of the fungus, Scedosporium related subcutaneous infections reported in the past quarter of century in European countries were also reviewed. Corticosteroid treatment represents a serious risk factor of S. apiospermum infections, especially if the patient get in touch with manure-enriched or polluted soil or water. Such infections have emerged several times in European countries in the past decades. The presented data suggest that besides the commonly applied voriconazole, terbinafine may be an alternative for the therapy of mycetomatous Scedosporium infections.

[Evaluation of stents in treating childhood benign esophageal strictures].

PubMed

Reinshagen, K; Kähler, G; Manegold, B C; Waag, K-L

2009-01-01

Esophageal stenting is a popular of treatment of esophageal strictures in adults. It has also been described for children with benign strictures who did not respond to standard dilatation therapy. The aim of the study was to evaluate weather esophageal stents could be used safely and effectively in the treatment of benign esophageal strictures in children. From 1993 to 2005 stenting therapy was performed in 12 children with complicated esophageal strictures. Etiologies of the strictures were caustic burns in 9 patients, postoperative strictures due to complicated esophageal atresia in 2 patients and iatrogenic esophageal injury in 1 patient. Esophageal silicon tubi, covered retrievable expandable nitinol and plastic stents were placed endoscopically. The clinical course and the long term follow up were evaluated retrospectively The stents and tubi were placed in all patients without complications and were later removed successfully. 6 patients were treated with a self expanding plastic stent. The plastic stents showed a distinct tendency to migrate but in 5/6 patients esophageal stricture was treated successfully. 3 patients were treated by a covered self expanding nitinol stent. No migration occurred. One patient was asymptomatic after therapy, one required further dilatation therapy and the third had esophageal resection. 3 patients were treated by esophageal tubi. 2 patients required surgery in the follow up, one patient is asymptomatic. The use of stenting devices in children to treat benign esophageal strictures is safe and efficient. The self expanding plastic stents had the best long term results but required high compliance of parents and children due to the tendency of stent migration. Self expanding nitinol stents are more traumatic at the extraction procedure and are useful in patients with low compliance. Recurrence of strictures occurred most often after esophageal tubi possibly due to the lack of radial expansion.

Thirty-eight cases of Bartholin's cyst treated with laser

NASA Astrophysics Data System (ADS)

Jia, Zhenguo

1993-03-01

Bartholin's cyst is a common disease of gynecology. It is cystic expansion of the gland tubes, which is caused by scar and infection. Forty years ago this disease was completely cut away by operation. In 1966, someone thought that cystostomy was a certain treatment method. Although there are many therapies used to treat this disease, e.g., incision drainage, excision, puncture, etc., they all have a better or worse side. In the past two years we used CO2 laser to treat patients in our hospital. Comparing outpatients with inpatients, the writer thinks that inpatients treated with laser (CO2) have less bleeding, less pain, faster recovery, and a higher success rate. This operation is done in the clinic and its both convenient and cheap. This method is one of the valuable methods which is worth spreading. From April 1989 to April 1991, 74 patients were treated with traditional operation and drug treatment. Another 38 patients were treated with the laser in the clinic. Clinical treatment results are contained here.

Erosive pustular dermatosis of the scalp successfully treated with oral prednisone and topical tacrolimus*

PubMed Central

Zahdi, Mariana Ribas; Seidel, Gabriela Bestani; Soares, Vanessa Cristina; de Freitas, Camila Fernanda Novak Pinheiro; Mulinari-Brenner, Fabiane Andrade

2013-01-01

Erosive pustular dermatosis of the scalp is a rare inflammatory disorder of the scalp, affecting elderly patients after local trauma and leading to scarring or cicatricial alopecia. Case Report: An elderly female patient complained of painful pustules on the parietal region bilaterally with progressive enlargement and ulceration. A biopsy suggested erosive pustular dermatosis of the scalp and the patient was treated with prednisone 40 mg/day and 0.1% topical tacrolimus. After 10 weeks complete closure of the eroded areas was observed and a stable scarring alopecia developed. PMID:24173187

Predictors of Functional Dependence Despite Successful Revascularization in Large-Vessel Occlusion Strokes

PubMed Central

Shi, Zhong-Song; Liebeskind, David S.; Xiang, Bin; Ge, Sijian Grace; Feng, Lei; Albers, Gregory W.; Budzik, Ronald; Devlin, Thomas; Gupta, Rishi; Jansen, Olav; Jovin, Tudor G.; Killer-Oberpfalzer, Monika; Lutsep, Helmi L.; Macho, Juan; Nogueira, Raul G.; Rymer, Marilyn; Smith, Wade S.; Wahlgren, Nils; Duckwiler, Gary R.

2014-01-01

Background and Purpose High revascularization rates in large-vessel occlusion strokes treated by mechanical thrombectomy are not always associated with good clinical outcomes. We evaluated predictors of functional dependence despite successful revascularization among patients with acute ischemic stroke treated with thrombectomy. Methods We analyzed the pooled data from the Multi Mechanical Embolus Removal in Cerebral Ischemia (MERCI), Thrombectomy Revascularization of Large Vessel Occlusions in Acute Ischemic Stroke (TREVO), and TREVO 2 trials. Successful revascularization was defined as thrombolysis in cerebral infarction score 2b or 3. Functional dependence was defined as a score of 3 to 6 on the modified Rankin Scale at 3 months. We assessed relationship of demographic, clinical, angiographic characteristics, and hemorrhage with functional dependence despite successful revascularization. Results Two hundred and twenty-eight patients with successful revascularization had clinical outcome follow-up. The rates of functional dependence with endovascular success were 48.6% for Trevo thrombectomy and 58.0% for Merci thrombectomy. Age (odds ratio, 1.04; 95% confidence interval, 1.02–1.06 per 1-year increase), National Institutes of Health Stroke Scale score (odds ratio, 1.08; 95% confidence interval, 1.02–1.15 per 1-point increase), and symptom onset to endovascular treatment time (odds ratio, 1.11; 95% confidence interval, 1.01–1.22 per 30-minute delay) were predictors of functional dependence despite successful revascularization. Symptom onset to reperfusion time beyond 5 hours was associated with functional dependence. All subjects with symptomatic intracranial hemorrhage had functional dependence. Conclusions One half of patients with successful mechanical thrombectomy do not have good outcomes. Age, severe neurological deficits, and delayed endovascular treatment were associated with functional dependence despite successful revascularization. Our data

The Parameters Affecting the Success of Irrigation and Debridement with Component Retention in the Treatment of Acutely Infected Total Knee Arthroplasty

PubMed Central

Kim, Jae Gyoon; Bae, Ji Hoon; Lee, Seung Yup; Cho, Won Tae

2015-01-01

Background The aims of our study were to evaluate the success rate of irrigation and debridement with component retention (IDCR) for acutely infected total knee arthroplasty (TKA) (< 4 weeks of symptom duration) and to analyze the factors affecting prognosis of IDCR. Methods We retrospectively reviewed 28 knees treated by IDCR for acutely infected TKA from 2003 to 2012. We evaluated the success rate of IDCR. All variables were compared between the success and failure groups. Multivariable logistic regression analysis was also used to examine the relative contribution of these parameters to the success of IDCR. Results Seventeen knees (60.7%) were successfully treated. Between the success and failure groups, there were significant differences in the time from primary TKA to IDCR (p = 0.021), the preoperative erythrocyte sedimentation rate (ESR; p = 0.021), microorganism (p = 0.006), and polyethylene liner exchange (p = 0.017). Multivariable logistic regression analysis of parameters affecting the success of IDCR demonstrated that preoperative ESR (odds ratio [OR], 1.02; p = 0.041), microorganism (OR, 12.4; p = 0.006), and polyethylene liner exchange (OR, 0.07; p = 0.021) were significant parameters. Conclusions The results show that 60.7% of the cases were successfully treated by IDCR for acutely infected TKA. The preoperative ESR, microorganism, and polyethylene liner exchange were factors that affected the success of IDCR in acutely infected TKA. PMID:25729521

Stem Cell Therapy to Treat Diabetes Mellitus

PubMed Central

Liew, Chee Gee; Andrews, Peter W.

2008-01-01

Transplantation of pancreatic islets offers a direct treatment for type 1 diabetes and in some cases, insulin-dependent type 2 diabetes. However, its widespread use is hampered by a shortage of donor organs. Many extant studies have focused on deriving β-cell progenitors from pancreas and pluripotent stem cells. Efforts to generate β-cells in vitro will help elucidate the mechanisms of β-cell formation and thus provide a versatile in vivo system to evaluate the therapeutic potential of these cells to treat diabetes. Various successful experiments using β-cells in animal models have generated extensive interest in using human embryonic stem cells to restore normoglycemia in diabetic patients. While new techniques are continually unveiled, the success of β-cell generation rests upon successful manipulation of culture conditions and the induction of key regulatory genes implicated in pancreas development. In this review, we compare successfully conducted protocols, highlight essential steps and identify some of the remarkable shortfalls common to these methods. In addition, we discuss recent advancements in the derivation of patient-specific pluripotent stem cells that may facilitate the use of autologous β-cells in stem cell therapy. PMID:19290381

Clinical outcome of radiosynoviorthesis: a meta-analysis including 2190 treated joints.

PubMed

Kresnik, E; Mikosch, P; Gallowitsch, H J; Jesenko, R; Just, H; Kogler, D; Gasser, J; Heinisch, M; Unterweger, O; Kumnig, G; Gomez, I; Lind, P

2002-07-01

A variety of indications for radiosynoviorthesis have been reported in literature, but the clinical outcome differs and depends on the primary disease and the pre-existing degenerative changes. This metaanalysis was carried out to establish groups for radiosynoviorthesis based on clinical outcome with respect to primary disease, clinical stage and the pre-existing degenerative changes. The literature search was carried out using the MEDLINE search term 'radionuclide synovectomy'. Based on reports in the literature we determined groups for radiosynoviorthesis for clinical use. Our literature list comprised 2190 joints that were treated with radiosynoviorthesis. The overall response rate for all treated joints was 72.5+/-17%. The mean improvement rate for the treated joints in rheumatoid arthritis was 66.7+/-15.4%. For osteoarthritis the success rate was 56+/-11%, with better results in case of minimal radiological changes. Radiosynoviorthesis in patients with changes according to Steinbrocker I and II was successful in 72.8+/-12.3%, and in 64+/-17.3%, respectively. Steinbrocker III and IV had a mean success rate of 52.4+/-23.6%. In the case of haemophilia and Willebrand's disease a reduction of joint bleedings and factor usages after radiosynoviorthesis was evident in 91+/-4.3%. In patients with pigmented villonodular synovitis radiosynoviorthesis was successful in 77.3+/-25.3%. It is concluded that radiosynoviorthesis provides better results in rheumatoid arthritis than in osteoarthritis. Minimal or moderate changes according to Steinbrocker stages I and II respond better to radionuclide therapy than do stages III and IV. Deformed or unstable joints might fail treatment and therefore surgical interventions should be considered. Close cooperation with orthopaedists and rheumatologists is necessary to consider radiosynoviorthesis in each patient to ensure optimal medical care.

Peri-stent aneurysm formation following a stent implant for stenotic intracranial vertebral artery dissection: a technical report of two cases successfully treated with coil embolization.

PubMed

Ishimaru, Hideki; Nakashima, Kazuaki; Takahata, Hideaki; Matsuoka, Yohjiro

2013-02-01

Although stenting for stenotic vertebral artery dissection (VAD) improves compromised blood flow, subsequent peri-stent aneurysm (PSA) formation is not well-known. We report two cases with PSA successfully treated with coil embolization. Three patients with stenotic intracranial VAD underwent endovascular angioplasty at our institution because they had acute infarction in posterior circulation territory and clinical evidence of hemodynamic insufficiency. In two of three patients balloon angioplasty at first session failed to relieve the stenosis, and a coronary stent was implanted. Angiography immediately after stenting showed no abnormality in case 1 and minimal slit-like projection at proximal portion of the stent in case 2. Angiography obtained 16 months after the stenting revealed PSA in case 1. In case 2, angiography performed 3 months later showed that the projection at proximal portion enlarged and formed an aneurysm outside the stent. Because follow-up angiographies showed growth of the aneurysm in both cases, endovascular aneurysmal embolization was performed. We advanced a microcatheter into the aneurysm through the strut of existing stent and delivered detachable coils into the aneurysm lumen successfully in both cases. The post-procedural course was uneventful, and complete obliteration of aneurysm was confirmed on angiography in both cases. Stenting for stenotic intracranial VAD may result in delayed PSA; therefore, follow-up angiographies would be necessary after stenting for stenotic intracranial arterial dissection. Coil embolization through the stent strut would be a solution for enlarging PSA.

Orchiopexy for intra-abdominal testes: factors predicting success.

PubMed

Stec, Andrew A; Tanaka, Stacy T; Adams, Mark C; Pope, John C; Thomas, John C; Brock, John W

2009-10-01

Intra-abdominal testes can be treated with several surgical procedures. We evaluated factors influencing the outcome of orchiopexy for intra-abdominal testis. We retrospectively reviewed 156 consecutive orchiopexies performed for intra-abdominal testis, defined as a nonpalpable testis on examination and located in the abdomen at surgery. All surgical approaches were included in the study. Primary outcome was the overall success rate and secondary outcomes were success based on surgical approach, age and a patent processus vaginalis. Success was considered a testis with normal texture and size compared to the contralateral testis at followup. Multivariate analysis was performed to determine factors predictive of success. The overall success rate of all orchiopexies was 79.5%. Median patient age at orchiopexy was 12 months and mean followup was 16 months. Of the patients 117 had a patent processus vaginalis at surgery. One-stage abdominal orchiopexy was performed in 92 testes with 89.1% success. Of these cases 32 were performed laparoscopically with 96.9% success. One-stage Fowler-Stephens orchiopexy was performed in 27 testes and 2-stage Fowler-Stephens orchiopexy was performed in 37 with success in 63.0% and 67.6%, respectively. Multivariate analysis revealed that 1-stage orchiopexy without vessel division had more successful outcomes than 1 and 2-stage Fowler-Stephens orchiopexy (OR 0.24, p = 0.007 and 0.29, p = 0.19, respectively). Neither age at surgery nor an open internal ring was significant (p = 0.49 and 0.12, respectively). The overall success of orchiopexy for intra-abdominal testis is 79.5%. While patient selection remains a critical factor, 1-stage orchiopexy without vessel division was significantly more successful and a laparoscopic approach was associated with the fewest failures for intra-abdominal testes.

Use of a lumen-apposing metal stent to treat GI strictures (with videos).

PubMed

Irani, Shayan; Jalaj, Sujai; Ross, Andrew; Larsen, Michael; Grimm, Ian S; Baron, Todd H

2017-06-01

Benign GI strictures occur typically in the esophagus and pyloric channel but can occur anywhere in the GI tract and at anastomotic sites. Such strictures can be treated with dilation, incisional therapy, steroid injection, and stents. Our aim was to describe the use of a lumen-apposing metal stent (LAMS) to treat short, benign GI strictures. Consecutive patients who underwent LAMS placement for various benign strictures at 2 tertiary care centers from August 2014 to November 2015 were reviewed retrospectively. The main outcome measures were technical success, clinical success, stent migration, and adverse events. Twenty-five patients (7 males, 18 females) with a median age of 54 years (33-85 years) underwent 28 LAMS placements to treat various benign strictures. The location of the strictures included esophagogastric anastomoses (n=4), gastrojejunal anastomoses (n=13), pylorus (n=6), vertical banded gastroplasty (n=1), and ileocolonic anastomosis (n=1). Twenty patients had been previously treated with dilation alone (9 patients with ≥3 dilations), 11 patients with dilation and steroid injection, 2 patients with additional needle-knife therapy, and 1 patient with placement of a traditional fully covered self-expandable metal stent. A 15-mm internal diameter LAMS was placed in all patients; 3 patients had been treated previously with a 10-mm LAMS. Technical success was achieved in all patients, whereas clinical success was achieved in 15 of 25 patients (60%) who completed a minimum of 6 months of follow-up after placement. Median stent dwell time was 92 days (range, 3-273 days). Stent migration was seen in 2 of 28 stent placements (7%). Four of 25 patients (16%) developed 5 moderate adverse events (pain requiring removal, 2; new stricture formation, 2; bleeding, 1). Median follow-up was 301 days after stent placement. Study limitations include the small, select group of patients, the retrospective study design, and short follow-up. LAMS placement for benign GI

Prognostic factors for non-success in patients with sciatica and disc herniation.

PubMed

Haugen, Anne Julsrud; Brox, Jens Ivar; Grøvle, Lars; Keller, Anne; Natvig, Bård; Soldal, Dag; Grotle, Margreth

2012-09-22

Few studies have investigated prognostic factors for patients with sciatica, especially for patients treated without surgery. The aim of this study was to identify factors associated with non-success after 1 and 2 years of follow-up and to test the prognostic value of surgical treatment for sciatica. The study was a prospective multicentre observational study including 466 patients with sciatica and lumbar disc herniation. Potential prognostic factors were sociodemographic characteristics, back pain history, kinesiophobia, emotional distress, pain, comorbidity and clinical examination findings. Study participation did not alter treatment considerations for the patients in the clinics. Patients reported on the questionnaires if surgery of the disc herniation had been performed. Uni- and multivariate logistic regression analyses were used to evaluate factors associated with non-success, defined as Maine-Seattle Back Questionnaire score of ≥5 (0-12) (primary outcome) and Sciatica Bothersomeness Index ≥7 (0-24) (secondary outcome). Rates of non-success were at 1 and 2 years 44% and 39% for the main outcome and 47% and 42% for the secondary outcome. Approximately 1/3 of the patients were treated surgically. For the main outcome variable, in the final multivariate model non-success at 1 year was significantly associated with being male (OR 1.70 [95% CI; 1.06 - 2.73]), smoker (2.06 [1.31 - 3.25]), more back pain (1.0 [1.01 - 1.02]), more comorbid subjective health complaints (1.09 [1.03 - 1.15]), reduced tendon reflex (1.62 [1.03 - 2.56]), and not treated surgically (2.97 [1.75 - 5.04]). Further, factors significantly associated with non-success at 2 years were duration of back problems >; 1 year (1.92 [1.11 - 3.32]), duration of sciatica >; 3 months (2.30 [1.40 - 3.80]), more comorbid subjective health complaints (1.10 [1.03 - 1.17]) and kinesiophobia (1.04 [1.00 - 1.08]). For the secondary outcome variable

Creosote movement from treated wood immersed in fresh water

Treesearch

Sung-Mo Kang; Jeffrey J. Morrell; John Simonsen; Stan Lebow

2005-01-01

Creosote has a long history of successful use as a wood preservative, but polycyclic aromatic hydrocarbons in this preservative have raised environmental concerns, particularly when creosote-treated wood is used in aquatic environments. A number of models have been developed to predict the risk of creosote use in aquatic environments, but one limitation of these models...

Successful percutaneous transperineal drainage of a large prostatic abscess.

PubMed

Mason, Barry M; Hakimi, A Ari; Clerkin, Kevin J; Silva, Jose V

2010-12-01

We present a case of an 83-year-old man with septic shock secondary to an extremely large prostatic abscess. Antibiotics and transperineal percutaneous drainage with a suprapubic-type Malecot catheter successfully treated the abscess. Follow-up images reveal resolution of the abscess. Broad-spectrum antibiotics and drainage permitted a full recovery. Copyright © 2010 Elsevier Inc. All rights reserved.

Effects of heptachlor- and lindane-treated seed on Canada geese

USGS Publications Warehouse

Blus, L.J.; Henny, C.J.; Lenhart, D.J.; Kaiser, T.E.

1984-01-01

A study of Canada geese (B. canadensis) was conducted in the Pacific Northwest from 1978 through 1981. Lowered reproductive success, mortality of adults, and a population decline of resident western Canada geese (B. c. moffitti) at the Umatilla National Wildlife Refuge (Umatilla NWR) Oregon and Washington [USA] were associated with the use of heptachlor-treated wheat. Analyses of eggs and tissues from dead geese provided strong evidence that heptachlor was responsible for the mortality and decreased production. Residues of heptachlor epoxide (HE) in brains of B. c. moffitti found dead in 1978 and 1979 equalled or exceeded the lethal hazard zone of 8-9 .mu.g/g in experimental passerine birds; HE residues of > 10 .mu.g/g in sample eggs were associated with low nest success. The breeding population of Canada geese at Umatilla decreased from 129 pairs in 1974 to about 100 pairs in 1979. The heptachlor problem was restricted to the Umatilla area; HE residues were low in tissues and eggs collected at other sites in Oregon, Washington and Idaho. In Sept., 1979, the use of heptachlor-treated seed was banned in a 1700-km2 area that encompassed both sides of the Columbia River near Umatilla. Lindane was substituted for heptachlor in the restricted area in 1979, and its use was extended to much of the Columbia Basin in 1981. Concurrently, reproductive success of geese increased, mortality decreased, and the nesting population increased to 170 pairs by 1983. There was no evidence for either biomagnification of lindance residues from treated seed to goose tissues or eggs or for induction of adverse effects by this compound.

Bedside talc pleurodesis for malignant pleural effusion: factors affecting success.

PubMed

Aydogmus, Umit; Ozdemir, Servet; Cansever, Levent; Sonmezoglu, Yasar; Kocaturk, Celalettin Ibrahim; Bedirhan, Mehmet Ali

2009-03-01

To determine the factors affecting the success of bedside talc slurry (TS) used for symptomatic treatment of patients with malignant pleural effusion (MPE). Data of 113 effusions in 103 MPE patients treated between 1999 and 2007 were retrospectively evaluated for the study. The study group involved 73 patients whose follow-up information was available out of 81 patients treated by TS. Causes of MPE were lung cancer in 22 patients (30.1%) and breast carcinoma in 21 patients (28.8%). The success rate of TS was significantly higher if the time period between radiological diagnosis of effusion and administration of TS was less than 30 days (P= .02), or spontaneous expansion was attained after chest tube drainage (CTD) (P= .01). Success rate was higher for patients with daily drainage of less than 200 ml before TS than patients with more than 200 ml of daily drainage (P= .01). Dose of talc, either 4 g or above (P= .34), primary cause of MPE (P= .53), time to termination of CTD (P= .57), amount of drainage when CTD was terminated (P= .23), and time period between CTD and administration of TS (P= .20) did not show a statistically significant effect on the success of TS. In the treatment of malignant pleural effusion, patients with daily drainage of less than 200 ml before TS developed less recurrence than patients with daily drainage of more than 200 ml. Longer time period between the diagnosis of MPE and onset of CTD increased recurrence.

Inverse association between brown adipose tissue activation and white adipose tissue accumulation in successfully treated pediatric malignancy1234

PubMed Central

Chalfant, James S; Smith, Michelle L; Hu, Houchun H; Dorey, Fred J; Goodarzian, Fariba; Fu, Cecilia H

2012-01-01

Background: Although the accumulation of white adipose tissue (WAT) is a risk factor for disease, brown adipose tissue (BAT) has been suggested to have a protective role against obesity. Objective: We studied whether changes in BAT were related to changes in the amounts of subcutaneous adipose tissue (SAT) and visceral adipose tissue (VAT) in children treated for malignancy. Design: We examined the effect of BAT activity on weight, SAT, and VAT in 32 pediatric patients with cancer whose positron emission tomography–computed tomography (PET-CT) scans at diagnosis showed no BAT activity. Changes in weight, SAT, and VAT from diagnosis to remission for children with metabolically active BAT at disease-free follow-up (BAT+) were compared with those in children without visualized BAT when free of disease (BAT−). Results: Follow-up PET-CT studies (4.7 ± 2.4 mo later) after successful treatment of the cancer showed BAT+ in 19 patients but no active BAT (BAT−) in 13 patients. BAT+ patients, in comparison with BAT− patients, gained significantly less weight (3.3 ± 6.6% compared with 11.0 ± 11.6%; P = 0.02) and had significantly less SAT (18.2 ± 26.5% compared with 67.4 ± 71.7%; P = 0.01) and VAT (22.6 ± 33.5% compared with 131.6 ± 171.8%; P = 0.01) during treatment. Multiple regression analysis indicated that the inverse relations between BAT activation and measures of weight, SAT, and VAT persisted even after age, glucocorticoid treatment, and the season when the PET-CT scans were obtained were accounted for. Conclusion: The activation of BAT in pediatric patients undergoing treatment of malignancy is associated with significantly less adipose accumulation. This trial was registered at clinicaltrials.gov as NCT01517581. PMID:22456659

Spontaneous Hemothorax in Neurofibromatosis Treated with Percutaneous Embolization

DOE Office of Scientific and Technical Information (OSTI.GOV)

Arai, Kazunori; Sanada, Junichiro; Kurozumi, Akiko

We evaluated the effectiveness of transcatheter arterial coil embolization therapy for the treatment of spontaneous hemothorax followed by aneurysm rupture in neurofibromatosis patients. Three patients were treated for massive hemothorax caused by arterial lesions associated with neurofibromatosis. Bleeding episodes were secondary to ascending cervical artery aneurysm and dissection of vertebral artery in 1 patient, and intercostal artery aneurysm with or without arteriovenous fistula in 2 patients. Patients were treated by transarterial coil embolization combined with chest drainage. In 1 patient, the ruptured ascending cervical artery aneurysm was well embolized but, shortly after the embolization, fatal hemorrhage induced by dissection ofmore » the vertebral artery occurred and the patient died. In the other 2 patients, the ruptured intercostal artery aneurysm was well embolized and they were successfully treated and discharged. Transcatheter arterial coil embolization therapy is an effective method for the treatment of spontaneous hemothorax followed by aneurysm rupture in neurofibromatosis patients.« less

Endoscopic mucosal autograft for treating esophageal caustic strictures: preliminary human experience.

PubMed

He, Kexin; Zhao, Lili; Bu, Shoushan; Liu, Li; Wang, Xiang; Wang, Min; Fan, Zhining

2018-06-11

 Esophageal caustic stricture is a stubborn disease and postoperative restenosis limits the clinical efficacy of endoscopic dilation. Autologous mucosal grafts have been successfully applied in the treatment of urethral stricture and in the prevention of stricture after extensive mucosal resection. We aimed to use mucosal autografting performed endoscopically to treat refractory esophageal stricture. METHODS : Three patients with intractable corrosive esophageal stricture were treated endoscopically by combining dilation with autologous mucosal transplantation. RESULTS : All procedures were successful with no severe complications. Mucosal regeneration was shown at the transplanted segments. One patient was able to maintain a normal diet with complete remission after 1 year of follow-up. Intraluminal stenosis and dysphagia were significantly improved in another two patients. CONCLUSIONS : Mucosal autografting can achieve esophageal re-epithelialization, inhibit undesired fibrosis, prevent restenosis, and promote functional regeneration. © Georg Thieme Verlag KG Stuttgart · New York.

Association between hemodynamic modifications and clinical outcome of intracranial aneurysms treated using flow diverters

NASA Astrophysics Data System (ADS)

Paliwal, Nikhil; Damiano, Robert J.; Davies, Jason M.; Siddiqui, Adnan H.; Meng, Hui

2017-03-01

Treatment of intracranial aneurysms (IAs) has been revolutionized by the advent of endovascular Flow Diverters (FDs), which disrupt blood flow within the aneurysm to induce pro-thrombotic conditions, and serves as a scaffold for endothelial ingrowth and arterial remodeling. Despite good clinical success of FDs, complications like incomplete occlusion and post-treatment rupture leading to subarachnoid hemorrhage have been reported. In silico computational fluid dynamic analysis of the pre- and post-treated geometries of IA patients can shed light on the contrasting blood hemodynamics associated with different clinical outcomes. In this study, we analyzed hemodynamic modifications in 15 IA patients treated using a single FD; 10 IAs were completely occluded (successful) and 5 were partially occluded (unsuccessful) at 12-month follow-up. An in-house virtual stenting workflow was used to recapitulate the clinical intervention on these cases, followed by CFD to obtain pre- and post-treatment hemodynamics. Bulk hemodynamic parameters showed comparable reductions in both groups with average inflow rate and aneurysmal velocity reduction of 40.3% and 52.4% in successful cases, and 34.4% and 49.2% in unsuccessful cases. There was a substantial reduction in localized parameter like vortex coreline length and Energy Loss for successful cases, 38.2% and 42.9% compared to 10.1% and 10.5% for unsuccessful cases. This suggest that for successfully treated IAs, the localized complex blood flow is disrupted more prominently by the FD as compared to unsuccessful cases. These localized hemodynamic parameters can be potentially used in prediction of treatment outcome, thus aiding the clinicians in a priori assessment of different treatment strategies.

Association between hemodynamic modifications and clinical outcome of intracranial aneurysms treated using flow diverters.

PubMed

Paliwal, Nikhil; Damiano, Robert J; Davies, Jason M; Siddiqui, Adnan H; Meng, Hui

2017-02-11

Treatment of intracranial aneurysms (IAs) has been revolutionized by the advent of endovascular Flow Diverters (FDs), which disrupt blood flow within the aneurysm to induce pro-thrombotic conditions, and serves as a scaffold for endothelial ingrowth and arterial remodeling. Despite good clinical success of FDs, complications like incomplete occlusion and post-treatment rupture leading to subarachnoid hemorrhage have been reported. In silico computational fluid dynamic analysis of the pre- and post-treated geometries of IA patients can shed light on the contrasting blood hemodynamics associated with different clinical outcomes. In this study, we analyzed hemodynamic modifications in 15 IA patients treated using a single FD; 10 IAs were completely occluded (successful) and 5 were partially occluded (unsuccessful) at 12-month follow-up. An in-house virtual stenting workflow was used to recapitulate the clinical intervention on these cases, followed by CFD to obtain pre- and post-treatment hemodynamics. Bulk hemodynamic parameters showed comparable reductions in both groups with average inflow rate and aneurysmal velocity reduction of 40.3% and 52.4% in successful cases, and 34.4% and 49.2% in unsuccessful cases. There was a substantial reduction in localized parameter like vortex coreline length and Energy Loss for successful cases, 38.2% and 42.9% compared to 10.1% and 10.5% for unsuccessful cases. This suggest that for successfully treated IAs, the localized complex blood flow is disrupted more prominently by the FD as compared to unsuccessful cases. These localized hemodynamic parameters can be potentially used in prediction of treatment outcome, thus aiding the clinicians in a priori assessment of different treatment strategies.

Congenital cytomegalovirus infection and Wiskott-Aldrich syndrome successfully treated with unrelated cord blood transplantation.

PubMed

Almagor, Yotam; Revel-Vilk, Shoshana; Averbuch, Diana; Mechoulam, Hadas; Engelhard, Dan; Resnick, Igor B; Weintraub, Michael; Stepensky, Polina

2011-10-01

We report a successful umbilical cord blood transplantation (UCBT) in an 8-month male with Wiskott-Aldrich syndrome (WAS) and congenital cytomegalovirus (CMV) infection. The child presented at 3 months of age with symptomatic thrombocytopenia and CMV infection. Despite appropriate antiviral treatment no rise in the platelet count was observed. Genetic analysis confirmed the diagnosis of WAS. The clinical course was complicated by severe CMV retinitis with bilateral retinal hemorrhages and renal vasculitis. He underwent unrelated UCBT resulting in a rapid resolution of autoimmunity and thrombocytopenia. Copyright © 2011 Wiley-Liss, Inc.

Successful living donor liver transplantation for classical maple syrup urine disease.

PubMed

Yasui, Toshihiro; Suzuki, Tatsuya; Hara, Fujio; Watanabe, Shunsuke; Uga, Naoko; Naoe, Atsuki; Yoshikawa, Tetsushi; Ito, Tetsuya; Nakajima, Yoko; Miura, Hiroki; Sugioka, Atsushi; Kato, Yutaro; Tokoro, Takamasa; Tanahashi, Yoshinao; Kasahara, Mureo; Fukuda, Akinari; Kurahashi, Hiroki

2016-08-01

MSUD is an autosomal recessive condition characterized by a deficiency in the enzyme, BCKDH, which catalyzes the breakdown of BCAAs. If left untreated, MSUD can result in mental retardation, central nervous system disorders, and even death. Most patients with MSUD are treated with a restricted protein diet and milk from which BCAAs have been removed. LT has been shown effective in patients with MSUD. This report describes the case of a 15-month-old boy who received a liver graft from his mother. Transplantation was successful, and the patient was then able to ingest a normal diet. Despite episodes of acute rejection, chylous ascites, and high fever (40 °C), he has shown no evidence of MSUD recurrence. These findings indicate that patients with MSUD can be successfully treated by LDLT, even when the donor is a heterozygous carrier of a mutated BCKDH gene. © 2016 The Authors. Pediatric Transplantation Published by Wiley Periodicals, Inc.

Operant Reinforcement with Structural Family Therapy in Treating Anorexia Nervosa

ERIC Educational Resources Information Center

Perlman, Lawrence M.; Bender, Sheila S.

1975-01-01

The authors successfully treated two families in which the index patients were anorectic adolescent girls by combining structural family therapy with behavioral modification techniques. Phases which appear to be typical of the treatment are described. A discussion of the value of the operant reinforcement procedure in family therapy follows.…

Forms of phosphorus transfer in runoff under no-tillage in a soil treated with successive swine effluents applications.

PubMed

Lourenzi, Cledimar Rogério; Ceretta, Carlos Alberto; Tiecher, Tadeu Luis; Lorensini, Felipe; Cancian, Adriana; Stefanello, Lincon; Girotto, Eduardo; Vieira, Renan Costa Beber; Ferreira, Paulo Ademar Avelar; Brunetto, Gustavo

2015-04-01

Successive swine effluent applications can substantially increase the transfer of phosphorus (P) forms in runoff. The aim of this study was to evaluate P accumulation in the soil and transfer of P forms in surface runoff from a Hapludalf soil under no-tillage subjected to successive swine effluent applications. This research was carried out in the Agricultural Engineering Department of the Federal University of Santa Maria, Brazil, from 2004 to 2007, on a Typic Hapludalf soil. Swine effluent rates of 0, 20, 40, and 80 m3 ha(-1) were broadcast over the soil surface prior to sowing of different species in a crop rotation. Soil samples were collected in stratified layers, and the levels of available P were determined. Samples of water runoff from the soil surface were collected throughout the period, and the available, soluble, particulate, and total P were measured. Successive swine effluent applications led to increases in P availability, especially in the soil surface, and P migration through the soil profile. Transfer of P forms was closely associated with runoff, which is directly related to rainfall volume. Swine effluent applications also reduced surface runoff. These results show that in areas with successive swine effluent applications, practices that promote higher water infiltration into the soil are required, e.g., crop rotation and no-tillage system.

Pleurovenous shunt for treating refractory benign pleural effusion.

PubMed

Bayram, Ahmet Sami; Köprücüoğlu, Mustafa; Aygün, Mert; Gebitekin, Cengiz

2008-05-01

We report the case of a 63-year-old female with hepatic cirrhosis due to chronic hepatitis C, successfully treated for refractory nonmalignant hepatic hydrothorax by using a long-term pleurovenous shunt (PVS). After failure of conventional treatment by mechanical pleurodesis, a PVS was inserted to drain the pleural fluid into the right subclavian vein. After 8 months of follow-up, the effusion is well controlled, and the shunt remains patent.

Studies of heat treated CSS CdS films

NASA Astrophysics Data System (ADS)

Marinskiy, D.; Marinskaya, S.; Viswanathan, V.; Morel, D. L.; Ferekides, C. S.

1999-03-01

Cadmium sulfide continues to be the most successful and widely used n-type heterojunction partner in thin film CdTe solar cells. In most cases solar cell performance is enhanced if the CdS films are heat treated prior to the deposition of the CdTe. This paper discusses the effect of H2 annealing on the resistivity of CSS-CdS films and the use of a mobility activation model to explain the observed changes in resistivity. Photoluminescence measurements of CSS CdS films heat-treated in He and in CdCl2 vapor have also been carried out. In all cases the heat treatments lead to an increase in the intensity of a photoluminescence band believed to be associated with sulfur vacancies.

CCG Programs with Clinical Data will Build on the Success of TCGA

Cancer.gov

Though TCGA will wrap up in 2016 with a concluding symposium, cancer genomics projects built upon the success of TCGA will continue to play a major part in the NCI’s mission to better understand and treat cancer in the years to come.

High-resolution single-nucleotide polymorphism array-profiling in myeloproliferative neoplasms identifies novel genomic aberrations

PubMed Central

Stegelmann, Frank; Bullinger, Lars; Griesshammer, Martin; Holzmann, Karlheinz; Habdank, Marianne; Kuhn, Susanne; Maile, Carmen; Schauer, Stefanie; Döhner, Hartmut; Döhner, Konstanze

2010-01-01

Single-nucleotide polymorphism arrays allow for genome-wide profiling of copy-number alterations and copy-neutral runs of homozygosity at high resolution. To identify novel genetic lesions in myeloproliferative neoplasms, a large series of 151 clinically well characterized patients was analyzed in our study. Copy-number alterations were rare in essential thrombocythemia and polycythemia vera. In contrast, approximately one third of myelofibrosis patients exhibited small genomic losses (less than 5 Mb). In 2 secondary myelofibrosis cases the tumor suppressor gene NF1 in 17q11.2 was affected. Sequencing analyses revealed a mutation in the remaining NF1 allele of one patient. In terms of copy-neutral aberrations, no chromosomes other than 9p were recurrently affected. In conclusion, novel genomic aberrations were identified in our study, in particular in patients with myelofibrosis. Further analyses on single-gene level are necessary to uncover the mechanisms that are involved in the pathogenesis of myeloproliferative neoplasms. PMID:20015882

A case of lung abscess successfully treated by transbronchial drainage using a guide sheath.

PubMed

Izumi, Hiroki; Kodani, Masahiro; Matsumoto, Shingo; Kawasaki, Yuji; Igishi, Tadashi; Shimizu, Eiji

2017-05-01

A 51-year-old man was diagnosed with colon cancer in September 2011, and a solitary pulmonary nodule was detected by computed tomography (CT) scan. We performed a transbronchial biopsy with endobronchial ultrasonography using a guide sheath (GS) and diagnosed lung metastasis of colon cancer. The patient experienced remittent fever after the biopsy in spite of intravenous antibiotic therapies. Moreover, his CT scan showed a large lung abscess at the biopsy site. We performed transbronchial drainage using a GS as salvage therapy. The bloody pus was successfully aspirated, and chest X-ray following the procedure showed dramatic shrinkage of the abscess.

Lyme neuroborreliosis in HIV-1 positive men successfully treated with oral doxycycline: a case series and literature review

PubMed Central

2011-01-01

Introduction Lyme neuroborreliosis is the most common bacterial central nervous system infection in the temperate parts of the northern hemisphere. Even though human immunodeficiency virus (HIV) -1 infection is common in Lyme borreliosis endemic areas, only five cases of co-infection have previously been published. Four of these cases presented with typical Lyme neuroborreliosis symptoms such as meningoradiculitis and facial palsy, while a fifth case had more severe symptoms of encephalomyelitis. All five were treated with intravenous cephalosporins and clinical outcome was good for all but the fifth case Case presentations We present four patients with concomitant presence of HIV-1 infection and Lyme neuroborreliosis diagnosed in Western Sweden. Patient 1 was a 60-year-old Caucasian man with radicular pain and cognitive impairment. Patient 2 was a 39-year-old Caucasian man with headaches, leg weakness, and pontine infarction. Patient 3 was a 62-year-old Caucasian man with headaches, tremor, vertigo, and normal-pressure hydrocephalus. Patient 4 was a 50-year-old Caucasian man with radicular pain and peripheral facial palsy. Patients one, two, and three all had subnormal levels of CD4 cells, indicating impaired immunity. All patients were treated with oral doxycycline with good clinical outcome and normalization of CSF pleocytosis. Conclusion Given the low HIV-1 prevalence and medium incidence of Lyme neuroborreliosis in Western Sweden where these four cases were diagnosed, co-infection with HIV-1 and Borrelia is probably more common than previously thought. The three patients that were the most immunocompromised suffered from more severe and rather atypical neurological symptoms than are usually described among patients with Lyme neuroborreliosis. It is therefore important for doctors treating HIV patients to consider Lyme neuroborreliosis in a patient presenting with atypical neurological symptoms. All four patients were treated with oral doxycycline with a good

Lyme neuroborreliosis in HIV-1 positive men successfully treated with oral doxycycline: a case series and literature review.

PubMed

Bremell, Daniel; Säll, Christer; Gisslén, Magnus; Hagberg, Lars

2011-09-19

Lyme neuroborreliosis is the most common bacterial central nervous system infection in the temperate parts of the northern hemisphere. Even though human immunodeficiency virus (HIV) -1 infection is common in Lyme borreliosis endemic areas, only five cases of co-infection have previously been published. Four of these cases presented with typical Lyme neuroborreliosis symptoms such as meningoradiculitis and facial palsy, while a fifth case had more severe symptoms of encephalomyelitis. All five were treated with intravenous cephalosporins and clinical outcome was good for all but the fifth case We present four patients with concomitant presence of HIV-1 infection and Lyme neuroborreliosis diagnosed in Western Sweden. Patient 1 was a 60-year-old Caucasian man with radicular pain and cognitive impairment. Patient 2 was a 39-year-old Caucasian man with headaches, leg weakness, and pontine infarction. Patient 3 was a 62-year-old Caucasian man with headaches, tremor, vertigo, and normal-pressure hydrocephalus. Patient 4 was a 50-year-old Caucasian man with radicular pain and peripheral facial palsy. Patients one, two, and three all had subnormal levels of CD4 cells, indicating impaired immunity. All patients were treated with oral doxycycline with good clinical outcome and normalization of CSF pleocytosis. Given the low HIV-1 prevalence and medium incidence of Lyme neuroborreliosis in Western Sweden where these four cases were diagnosed, co-infection with HIV-1 and Borrelia is probably more common than previously thought. The three patients that were the most immunocompromised suffered from more severe and rather atypical neurological symptoms than are usually described among patients with Lyme neuroborreliosis. It is therefore important for doctors treating HIV patients to consider Lyme neuroborreliosis in a patient presenting with atypical neurological symptoms. All four patients were treated with oral doxycycline with a good outcome, further proving the efficacy of

Prognostic factors for non-success in patients with sciatica and disc herniation

PubMed Central

2012-01-01

Background Few studies have investigated prognostic factors for patients with sciatica, especially for patients treated without surgery. The aim of this study was to identify factors associated with non-success after 1 and 2 years of follow-up and to test the prognostic value of surgical treatment for sciatica. Methods The study was a prospective multicentre observational study including 466 patients with sciatica and lumbar disc herniation. Potential prognostic factors were sociodemographic characteristics, back pain history, kinesiophobia, emotional distress, pain, comorbidity and clinical examination findings. Study participation did not alter treatment considerations for the patients in the clinics. Patients reported on the questionnaires if surgery of the disc herniation had been performed. Uni- and multivariate logistic regression analyses were used to evaluate factors associated with non-success, defined as Maine–Seattle Back Questionnaire score of ≥5 (0–12) (primary outcome) and Sciatica Bothersomeness Index ≥7 (0–24) (secondary outcome). Results Rates of non-success were at 1 and 2 years 44% and 39% for the main outcome and 47% and 42% for the secondary outcome. Approximately 1/3 of the patients were treated surgically. For the main outcome variable, in the final multivariate model non-success at 1 year was significantly associated with being male (OR 1.70 [95% CI; 1.06 − 2.73]), smoker (2.06 [1.31 − 3.25]), more back pain (1.0 [1.01 − 1.02]), more comorbid subjective health complaints (1.09 [1.03 − 1.15]), reduced tendon reflex (1.62 [1.03 − 2.56]), and not treated surgically (2.97 [1.75 − 5.04]). Further, factors significantly associated with non-success at 2 years were duration of back problems >; 1 year (1.92 [1.11 − 3.32]), duration of sciatica >; 3 months (2.30 [1.40 − 3.80]), more comorbid subjective health complaints (1.10 [1.03 − 1.17]) and kinesiophobia (1.04 [1

Behavioral Case Consultation with Parents and Teachers: An Example Using Differential Reinforcement to Treat Psychogenic Cough.

ERIC Educational Resources Information Center

Watson, T. Steuart; Heindl, Betsy

1996-01-01

Presents a demonstration of successful behavioral case consultation combined with case study methodology in the treatment of a 13-year-old female afflicted with a psychogenic cough. Results suggest that psychogenic cough can be successfully treated by using behavioral case consultation as a framework for applying intervention. Limitations are also…

Treating cerebral palsy with aculaser therapy

NASA Astrophysics Data System (ADS)

Anwar, Shahzad; Nazir Khan, Malik M.; Nadeem Khan, Malik M.; Qazi, Faiza M.; Awan, Abid H.; Dar, Irfan

2008-03-01

A single, open and non comparative study was conducted at Anwar Shah Trust for C.P. & Paralysis in collaboration with the Departments of Neurology and Neurosurgery, Children Hospital Lahore, Pakistan to evaluate the effects of ACULASER THERAPY in childern suffering from Cerebral Palsy (C.P.) and associated Neurological Disorders like epilepsy, cortical blindness, spasticity, hemiplegia, paraplegia, diplegia, quadriplegia, monoplegia, sensory-neural deafness and speech disorders. In all 250 childern were treated and the data was gathered during a period of 3 years from December 2003 till December 2006. These children were further classified according to the type of C.P. (spastic, athetoid, mixed) they suffered from and associated Neurological Disorders. This article shows results in C.P. childern who were treated with ACULASER THERAPY for minimum 6 weeks and more or had minimum of 15 treatment sessions and more. This article also shows that those childern who were given a break in the treatment for 1 month to 1 year did not show any reversal of the signs and symptoms. Analysis of the data showed that out of 171 children with Spasticity and Stiffness 147 showed marked improvement showing 87% success rate, out of 126 children with Epileptic fits, there was a significant reduction in the intensity, frequency and duration of Epileptic fits in 91 children showing 72% success rate, out of 48 children with Cortical Blindness 30 children showed improvement accounting for 63% efficacy rate, out of 105 children with Hearing Difficulties, 63 showed marked improvement accounting for 60% improvement rate, out of 190 children with Speech Disorders 122 showed improvement reflecting 64% improvement rate, out of 96 children with Hemiplegia 71 showed improvement in movement, tone and power accounting for 74% improvement rate, out of 76 children with Quadriplegia 52 showed improvement in gross and fine motor functions showing 69% success rate and out of 58 children with Paraplegia of

Full-mouth minimally invasive adhesive rehabilitation to treat severe dental erosion: a case report.

PubMed

Vailati, Francesca; Vaglio, Giovanna; Belser, Urs Christoph

2012-02-01

Dental erosion is increasing, and only recently are clinicians starting to acknowledge the problem. A prospective clinical trial investigating which therapeutic approach must be undertaken to treat erosion and when is under way at the University of Geneva (Geneva Erosion Study). All patients affected by dental erosion who present with signs of dentin exposure are immediately treated using only adhesive techniques. In this article, the full-mouth adhesive rehabilitation of one of these patients affected by severe dental erosion (ACE class IV) is illustrated. By the end of the therapy, a very pleasing esthetic outcome had been achieved (esthetic success), all of the patient's teeth maintained their vitality, and the amount of tooth structure sacrificed to complete the adhesive full-mouth rehabilitation was negligible (biological success).

Pilot study of the antifibrotic effects of the multikinase inhibitor pacritinib in a mouse model of liver fibrosis

PubMed Central

Al-Fayoumi, Suliman; Hashiguchi, Taishi; Shirakata, Yuka; Mascarenhas, John; Singer, Jack W

2018-01-01

Background Fibrotic diseases result from an exuberant response to chronic inflammation. Myelofibrosis is the end result of inflammation in bone, caused by an inflammatory process triggered by production of abnormal myeloid cells driven by mutations affecting the JAK–STAT pathway. Inflammatory cytokine overproduction leads to increased mesenchymal cell proliferation, culminating in fibrosis. Although JAK2 inhibitors, such as the JAK1/2 inhibitor ruxolitinib and the JAK2/FLT3/CSF1R/IRAK1 inhibitor pacritinib suppress abnormal clone expansion in myelofibrosis, ruxolitinib does not appear to prevent or reverse bone-marrow fibrosis in most patients. In two Phase III clinical trials, pacritinib, however, demonstrated improvements in platelet counts and hemoglobin and reductions in transfusion burden in some patients with baseline cytopenias, suggesting it may improve bone-marrow function. Unlike ruxolitinib, pacritinib suppresses signaling through IRAK1, a key control point for inflammatory and fibrotic signaling. Purpose To investigate potential antifibrotic effects of pacritinib in an animal model of liver fibrosis relevant to the observed course of human disease. Methods Pacritinib, negative control (vehicle), and positive control (the angiotensin 2-receptor antagonist and PPARγ partial agonist telmisartan) were assessed in the murine Stelic animal model, which mimics the clinically observed progression from hepatic steatosis to nonalcoholic steatohepatitis, liver fibrosis, and hepatocellular carcinoma. Histopathological analysis used hematoxylin and eosin staining. Body and liver weight changes, nonalcoholic fatty-liver disease activity scores, and plasma cytokeratin 18 fragment levels (a biomarker of hepatic necrosis) were measured. Results Pacritinib-treated mice had significantly (P<0.01) reduced fibrotic areas in liver compared to vehicle control and significantly (P<0.05) lower levels of CK18. The antifibrotic effect of pacritinib was comparable to that of

Pilot study of the antifibrotic effects of the multikinase inhibitor pacritinib in a mouse model of liver fibrosis.

PubMed

Al-Fayoumi, Suliman; Hashiguchi, Taishi; Shirakata, Yuka; Mascarenhas, John; Singer, Jack W

2018-01-01

Fibrotic diseases result from an exuberant response to chronic inflammation. Myelofibrosis is the end result of inflammation in bone, caused by an inflammatory process triggered by production of abnormal myeloid cells driven by mutations affecting the JAK-STAT pathway. Inflammatory cytokine overproduction leads to increased mesenchymal cell proliferation, culminating in fibrosis. Although JAK2 inhibitors, such as the JAK1/2 inhibitor ruxolitinib and the JAK2/FLT3/CSF1R/IRAK1 inhibitor pacritinib suppress abnormal clone expansion in myelofibrosis, ruxolitinib does not appear to prevent or reverse bone-marrow fibrosis in most patients. In two Phase III clinical trials, pacritinib, however, demonstrated improvements in platelet counts and hemoglobin and reductions in transfusion burden in some patients with baseline cytopenias, suggesting it may improve bone-marrow function. Unlike ruxolitinib, pacritinib suppresses signaling through IRAK1, a key control point for inflammatory and fibrotic signaling. To investigate potential antifibrotic effects of pacritinib in an animal model of liver fibrosis relevant to the observed course of human disease. Pacritinib, negative control (vehicle), and positive control (the angiotensin 2-receptor antagonist and PPARγ partial agonist telmisartan) were assessed in the murine Stelic animal model, which mimics the clinically observed progression from hepatic steatosis to nonalcoholic steatohepatitis, liver fibrosis, and hepatocellular carcinoma. Histopathological analysis used hematoxylin and eosin staining. Body and liver weight changes, nonalcoholic fatty-liver disease activity scores, and plasma cytokeratin 18 fragment levels (a biomarker of hepatic necrosis) were measured. Pacritinib-treated mice had significantly ( P <0.01) reduced fibrotic areas in liver compared to vehicle control and significantly ( P <0.05) lower levels of CK18. The antifibrotic effect of pacritinib was comparable to that of telmisartan, but without

Retrograde intrarenal surgery versus percutaneous lithotripsy to treat renal stones 2-3 cm in diameter.

PubMed

Zengin, Kursad; Tanik, Serhat; Karakoyunlu, Nihat; Sener, Nevzat Can; Albayrak, Sebahattin; Tuygun, Can; Bakirtas, Hasan; Imamoglu, M Abdurrahim; Gurdal, Mesut

2015-01-01

Retrograde intrarenal surgery (RIRS) performed using a flexible ureterorenoscope marked the beginning of a new era in urology. Today, even staghorn stones are successfully treated via RIRS. The recommended treatment for larger stones is percutaneous nephrolithotomy (PNL). However, the question of whether PNL or RIRS should be the first-line treatment option for larger stones remains controversial. In this study, we contribute to the debate by comparing the success and complication rates of PNL and RIRS that were used to treat renal pelvis stones 2-3 cm in diameter. The medical records of 154 patients (74 PNL, 80 RIRS) were retrospectively evaluated. PNL patients were placed in Group 1 and RIRS patients in Group 2. The complete stone-free rates were 95.5% in the PNL group and 80.6% in the RIRS group 1 month postoperatively (P = 0.061). The respective complication rates (evaluated using the Clavien system) were 13.5% and 8.8% (P = 0.520). RIRS affords a comparable success rate, causes fewer complications than PNL, and seems to be a promising alternative to PNL when larger stones are to be treated. Prospective randomized controlled trials are needed to confirm these findings.

Refractory immune thrombocytopenia successfully treated with high-dose vitamin D supplementation and hydroxychloroquine: two case reports

PubMed Central

2013-01-01

Introduction Immune thrombocytopenic purpura is thought to be characterized by an immune response against the host’s own platelets. If the thrombocytopenia is severe, patients are initially treated with high-dose steroids. Other more toxic second line treatments are considered if steroids fail. Here, we report the case of two patients in whom conventional treatment was unsuccessful but who responded to hydroxychloroquine and high-dose vitamin D replacement therapy. To the best of our knowledge, this is the first description of successful treatment for immune thrombocytopenia with high-dose vitamin D and hydroxychloroquine. Case presentation Case 1: We report the case of a 79-year-old Caucasian man who presented with high titer antinuclear antibodies, positive anti-SSA/Ro autoantibodies and clinically was felt to have an overlap of systemic lupus erythematosus and/or Sjögren’s syndrome with profound life-threatening thrombocytopenia. There was no evidence of underlying malignancy. The patient’s platelet count significantly increased with vitamin D and hydroxychloroquine treatment, but upon vitamin D discontinuation his platelet levels plummeted. Hydroxychloroquine therapy was maintained throughout treatment. With reinstitution of high-dose vitamin D therapy, platelet counts were restored to normal levels. Case 2: We also report the case of an 87-year-old Caucasian woman who presented with high titer antinuclear antibodies, positive anti-SSA/Ro autoantibodies and was felt to have an overlap of systemic lupus erythematosus and/or Sjögren’s syndrome with immune thrombocytopenia; she also had severely low levels of 25-hydroxy vitamin D (17ng/mL). There was no evidence of underlying malignancy. She responded to high-dose vitamin D replacement and hydroxychloroquine treatment, thereby alleviating the need for high-dose steroid treatment. She remains in remission while taking vitamin D, hydroxychloroquine and very low-dose prednisone. No untoward side effects

Quantitative MR assessment of longitudinal parenchymal changes in children treated for medulloblastoma

NASA Astrophysics Data System (ADS)

Reddick, Wilburn E.; Glass, John O.; Wu, Shingjie; Palmer, Shawna L.; Mulhern, Raymond K.; Gajjar, Amar

2002-05-01

Our research builds on the hypothesis that white matter damage, in children treated for cancer with cranial spinal irradiation, spans a continuum of severity that can be reliably probed using non-invasive MR technology and results in potentially debilitating neurological and neuropsychological problems. This longitudinal project focuses on 341 quantitative volumetric MR examinations from 58 children treated for medulloblastoma (MB) with cranial irradiation (CRT) of 35-40 Gy. Quadratic mixed effects models were used to fit changes in tissue volumes (white matter, gray matter, CSF, and cerebral) with time since CRT and age at CRT as covariates. We successfully defined algorithms that are useful in the prediction of brain development among children treated for MB.

Structural Brain Abnormalities in Successfully Treated HIV Infection: Associations With Disease and Cerebrospinal Fluid Biomarkers.

PubMed

van Zoest, Rosan A; Underwood, Jonathan; De Francesco, Davide; Sabin, Caroline A; Cole, James H; Wit, Ferdinand W; Caan, Matthan W A; Kootstra, Neeltje A; Fuchs, Dietmar; Zetterberg, Henrik; Majoie, Charles B L M; Portegies, Peter; Winston, Alan; Sharp, David J; Gisslén, Magnus; Reiss, Peter

2017-12-27

Brain structural abnormalities have been reported in persons living with human immunodeficiency virus (HIV; PLWH) who are receiving suppressive combination antiretroviral therapy (cART), but their pathophysiology remains unclear. We investigated factors associated with brain tissue volumes and white matter microstructure (fractional anisotropy) in 134 PLWH receiving suppressive cART and 79 comparable HIV-negative controls, aged ≥45 years, from the Comorbidity in Relation to AIDS cohort, using multimodal neuroimaging and cerebrospinal fluid biomarkers. Compared with controls, PLWH had lower gray matter volumes (-13.7 mL; 95% confidence interval, -25.1 to -2.2) and fractional anisotropy (-0.0073; 95% confidence interval, -.012 to -.0024), with the largest differences observed in those with prior clinical AIDS. Hypertension and the soluble CD14 concentration in cerebrospinal fluid were associated with lower fractional anisotropy. These associations were independent of HIV serostatus (Pinteraction = .32 and Pinteraction = .59, respectively) and did not explain the greater abnormalities in brain structure in relation to HIV infection. The presence of lower gray matter volumes and more white matter microstructural abnormalities in well-treated PLWH partly reflect a combination of historical effects of AIDS, as well as the more general influence of systemic factors, such as hypertension and ongoing neuroinflammation. Additional mechanisms explaining the accentuation of brain structure abnormalities in treated HIV infection remain to be identified. © The Author 2017. Published by Oxford University Press for the Infectious Diseases Society of America. All rights reserved. For permissions, e-mail: journals.permissions@oup.com.

Long-term negative impact on quality of life in patients with successfully treated Cushing's disease.

PubMed

Heald, Adrian H; Ghosh, Sandip; Bray, Stephanie; Gibson, Christine; Anderson, Simon G; Buckler, Helen; Fowler, Helen L

2004-10-01

A cohort of pituitary tumour patients, who had undergone definitive treatment within a 15-year period at a single neuroscience centre, were investigated as to whether there were differences in psychological well-being and psychosocial functioning, dependent on endocrine pathology and treatment variables. A detailed assessment of 114 patients with benign pituitary tumours in relation to primary diagnosis and mode of treatment was carried out. Psychological rating scales used were: the Hospital Anxiety and Depression Scale--UK version (HADS-UK), the World Health Organization Quality of Life Scale--abbreviated version (WHOQOL-BREF), General Health Questionnaire 28 (GHQ-28), the Functional Assessment of Cancer Therapy (FACT) and the Social Adjustment Scale--modified (SAS1 and SAS2; the former completed by the patient and the latter by another person who knows the patient well). All Cushing's patients were biochemically cured as defined by a normal 24-h urine free cortisol excretion within the previous 6 months. Patients with treated Cushing's disease had significantly impaired psychological well-being and psychosocial functioning across all tested domains compared with all other pituitary tumours, where scores were similar. When participants with Cushing's disease were excluded, patients who had undergone transfrontal surgery scored significantly higher on GHQ and HADS ratings than transsphenoidally treated patients. Cushing's disease even when biochemically stable has long-term adverse effects on mood and social functioning. We hypothesize that this may be related to irreversible changes in central neural function. Further studies are necessary to define the precise pathways involved.

Prophylactic Transcatheter Arterial Embolization After Successful Endoscopic Hemostasis in the Management of Bleeding Duodenal Ulcer.

PubMed

Mille, Markus; Huber, Juliane; Wlasak, Rüdiger; Engelhardt, Thomas; Hillner, Yvette; Kriechling, Henri; Aschenbach, Rene; Ende, Katrin; Scharf, Jens-Gerd; Puls, Ralf; Stier, Albrecht

2015-10-01

The aim of this study was to demonstrate the new strategy of prophylactic transcatheter arterial embolization (TAE) of the gastroduodenal artery after endoscopic hemostasis of bleeding duodenal ulcers. TAE is a well-established method for the treatment of recurrent or refractory ulcer bleeding resistant to endoscopic intervention, which increasingly replaces surgical procedures. A new approach for improving outcome and reducing rebleeding episodes is the supplemental and prophylactic TAE after successful endoscopic hemostasis. This retrospective study included all patients (n=117) treated from 2008 to 2012 for duodenal ulcer bleeding. After initial endoscopic hemostasis, patients were assessed regarding their individual rebleeding risk. Patients with a low rebleeding risk (n=47) were conservatively treated, patients with a high risk for rebleeding (n=55) had prophylactic TAE of the gastroduodenal artery, and patients with endoscopically refractory ulcer bleeding received immediate TAE. The technical success of prophylactic TAE was 98% and the clinical success was 87% of cases. Rebleeding occurred in 11% of patients with prophylactic TAE and was successfully treated with repeated TAE or endoscopy. The major complication rate was 4%. Surgery was necessary in only 1 prophylactic TAE patient (0.9%) during the whole study period. Mortality associated with ulcer bleeding was 4% in patients with prophylactic TAE. Prophylactic TAE in patients with duodenal ulcers at high risk for rebleeding was feasible, effective at preventing the need for surgery, and had low major complication rates. Given these promising outcomes, prophylactic TAE should be further evaluated as a preventative therapy in high-risk patients.

[A girl with self-harm treated with N-acetylcysteine (NAC)].

PubMed

Rus, C P

Deliberate and recurrent self-harm could be regarded as addictive behaviour that can be treated with medication. In addiction, the dopaminergic mesolimbic reward system is activated. Pain caused by cutting stimulates the reward system through the opioid system. Glutamatergic neurotransmission follows the same pathway and plays a role in addiction as well. In this case-study a 17-year-old girl was successfully treated with N-acetylcysteine (nac) in order to reduce the frequency of self-cutting. In addition, in this case nac reduced the symptoms of attention deficit/hyperactivity disorder and depression. nac modulates the glutamatergic neurotransmission. This article provides possible explanations for the effect of nac in this case.

Nanoparticles for imaging and treating brain cancer

PubMed Central

Meyers, Joseph D; Doane, Tennyson; Burda, Clemens; Basilion, James P

2013-01-01

Brain cancer tumors cause disruption of the selective properties of vascular endothelia, even causing disruptions in the very selective blood–brain barrier, which are collectively referred to as the blood–brain–tumor barrier. Nanoparticles (NPs) have previously shown great promise in taking advantage of this increased vascular permeability in other cancers, which results in increased accumulation in these cancers over time due to the accompanying loss of an effective lymph system. NPs have therefore attracted increased attention for treating brain cancer. While this research is just beginning, there have been many successes demonstrated thus far in both the laboratory and clinical setting. This review serves to present the reader with an overview of NPs for treating brain cancer and to provide an outlook on what may come in the future. For NPs, just like the blood–brain–tumor barrier, the future is wide open. PMID:23256496

Treating Panic Disorder Hypnotically.

PubMed

Reid, David B

2017-10-01

A hypnosis protocol for treating panic disorder is provided. The implementation of this protocol is demonstrated through a case example involving the successful treatment of a 28-year-old firefighter presenting with a 4-month history of near-daily panic attacks. Core principles associated with this protocol include: (1) Elementary education about the physiology of panic; (2) A review of primary factors contributing to the evolution and manifestation of panic; (3) Encouragement of physical activity; (4) Utilization of hypnosis applications; and (5) Monitoring and measuring progress evidenced by a reduction in the frequency and intensity of panic attacks. Six years after his last hypnosis session, "Jason," the once panicked firefighter returned to my office for concerns unrelated to panic, and reported that he remained panic-free, retained his job, and was twice promoted.

Sirolimus and Mycophenolate Mofetil in Preventing GVHD in Patients With Hematologic Malignancies Undergoing HSCT

ClinicalTrials.gov

2018-04-04

Adult Hodgkin Lymphoma; Adult Myelodysplastic Syndrome; Blast Phase Chronic Myelogenous Leukemia, BCR-ABL1 Positive; Childhood Chronic Myelogenous Leukemia, BCR-ABL1 Positive; Childhood Hodgkin Lymphoma; Childhood Myelodysplastic Syndrome; Chronic Phase Chronic Myelogenous Leukemia, BCR-ABL1 Positive; Myelofibrosis; Primary Myelofibrosis; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Adult Non-Hodgkin Lymphoma; Recurrent Childhood Acute Lymphoblastic Leukemia; Recurrent Childhood Acute Myeloid Leukemia; Recurrent Childhood Non-Hodgkin Lymphoma; Recurrent Chronic Myelogenous Leukemia, BCR-ABL1 Positive; Refractory Chronic Myelogenous Leukemia, BCR-ABL1 Positive; Refractory Non-Hodgkin Lymphoma

Soft tissue knee contracture of the knee due to melorheostosis, treated by total knee arthroplasty.

PubMed

Moulder, Elizabeth; Marsh, Clayton

2006-10-01

Melorheostosis is a rare condition which can cause soft tissue joint contractures. We present a case of melorheostosis causing disabling knee joint contracture, treated successfully by total knee arthroplasty.

Successful transradial intervention for two lesions with dual anomalous origins of coronary arteries.

PubMed

Masuda, Naoki; Matsukage, Takashi; Ikari, Yuji

2011-05-01

A 76-year-old male was admitted to our hospital for effort angina pectoris. His coronary computed tomography and coronary angiography revealed anomalous origins of the left anterior descending artery (LAD) from the proximal right coronary artery (RCA) and the left circumflex coronary artery (LCX) from the separate ostium in the right coronary cusp. Severe stenoses were present in the proximal segment of the LAD and in the middle segment of the LCX, which were successfully treated by 5 French (Fr) transradial approach intervention. Congenital coronary anomalies are relatively rare, with a prevalence of approximately 1.3% in a large-series study undergoing coronary angiography. Such anomalies occur in several anatomical arrangements, which have been classified in a number of research reports. Here we describe previously unreported dual anomalous origins of coronary arteries associated with significant atherosclerotic lesions, which were successfully treated by 5 Fr transradial approach intervention.

Sibling response to initial antiepileptic medication predicts treatment success.

PubMed

Ueda, Keisuke; Serajee, Fatema; Rajlich, Jan; Taraman, Sharief; Steckling, Lindsey; Huq, Ahm M

2017-10-01

A recent study focusing on a response to antiepileptic drugs (AED) among siblings for epilepsy showed a similar response among epileptic siblings to specific AEDs or AED combinations. Currently, however, family history of treatment response to AEDs is not readily employed in deciding which initial medication to use when treating patients with epilepsy. We tested the hypothesis that sibling response to initial AED predicts treatment success. Presumed siblings were identified from a single-center database of patients diagnosed with epilepsy by matching last name, address, and name of parent(s). We identified 28 sibling pairs and two sibling trios with epilepsy. Seventeen of these sibling pairs were started on the same initial AED, with 15 sibling pairs having the same type of epilepsy. The remaining 11 pairs were started on a different initial AED, with 8 of these sibling pairs having the same type of epilepsy. Subjects with seizure freedom for a period of ≥1year were classified as a "responder". When at least one of the sibling pair responded to an initial AED, the proportion of the other siblings also responding to the initial AED was significantly higher if the siblings were treated with the same AED (8/11) compared to siblings who were treated with different AED (1/10) (Fisher's exact test, p-value=0.0075). These findings suggest that sibling response to initial AED is predictive of the success of AED therapy. This study is limited by a small cohort and retrospective design. Future, larger prospective studies are needed to reproduce and further validate these findings. Copyright © 2017. Published by Elsevier B.V.

Successful surrogate pregnancy after ovarian transposition, pelvic irradiation and hysterectomy.

PubMed

Zinger, Michael; Liu, James H; Husseinzadeh, Nader; Thomas, Michael A

2004-07-01

Treatment of cervical cancer is often effective but at the cost of the woman's fertility. Ovarian transposition with subsequent oocyte retrieval and surrogate pregnancy can enable these patients to become genetic parents. We present the third reported such case. A 22-year-old woman was diagnosed with bulky, stage IB cervical cancer. Following transposition of both ovaries to the upper abdomen, she underwent pelvic irradiation followed by total abdominal hysterectomy. Eleven years later she presented for assisted reproduction. Two oocytes were retrieved following ovarian stimulation and transcutaneous, abdominal oocyte retrieval. One embryo was transferred to the gestational surrogate, resulting in a single intrauterine pregnancy and successful delivery at term. These procedures can preservefertility while successfully treating cervical cancer.

Reduced-Intensity Conditioning Before Donor Stem Cell Transplant in Treating Patients With High-Risk Hematologic Malignancies

ClinicalTrials.gov

2018-03-02

Accelerated Phase Chronic Myelogenous Leukemia; Adult Acute Lymphoblastic Leukemia in Remission; Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Nasal Type Extranodal NK/T-cell Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Blastic Phase Chronic Myelogenous Leukemia; Childhood Acute Lymphoblastic Leukemia in Remission; Childhood Burkitt Lymphoma; Childhood Chronic Myelogenous Leukemia; Childhood Diffuse Large Cell Lymphoma; Childhood Immunoblastic Large Cell Lymphoma; Childhood Myelodysplastic Syndromes; Childhood Nasal Type Extranodal NK/T-cell Lymphoma; Chronic Myelomonocytic Leukemia; Chronic Phase Chronic Myelogenous Leukemia; Cutaneous B-cell Non-Hodgkin Lymphoma; de Novo Myelodysplastic Syndromes; Essential Thrombocythemia; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Hepatosplenic T-cell Lymphoma; Intraocular Lymphoma; Juvenile Myelomonocytic Leukemia; Nodal Marginal Zone B-cell Lymphoma; Noncutaneous Extranodal Lymphoma; Peripheral T-cell Lymphoma; Polycythemia Vera; Post-transplant Lymphoproliferative Disorder; Previously Treated Myelodysplastic Syndromes; Primary Myelofibrosis; Recurrent Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Childhood Acute Myeloid Leukemia; Recurrent Childhood Anaplastic Large Cell Lymphoma; Recurrent Childhood Grade III Lymphomatoid Granulomatosis; Recurrent Childhood Large Cell Lymphoma; Recurrent Childhood Lymphoblastic Lymphoma; Recurrent Childhood Small Noncleaved Cell Lymphoma; Recurrent Cutaneous T-cell Non

A case of refractory chronic neutrophilic pustular folliculitis treated with adalimumab.

PubMed

Sand, Freja Laerke; Thomsen, Simon Francis

2015-01-01

Neutrophilic folliculitis is an often overlooked chronic condition characterized by a monomorphic eruption of "sterile" papulopustules. Neutrophilic folliculitis is often refractory to conventional treatment with topical and systemic antibiotics or isotretinoin. We report a case of severe pustular neutrophilic folliculitis successfully treated with the tumor necrosis factor-alpha inhibitor adalimumab. © 2014 Wiley Periodicals, Inc.

Neuromodulation by implant for treating lower urinary tract symptoms and dysfunction.

PubMed

Bemelmans, B L; Mundy, A R; Craggs, M D

1999-08-01

Patients with irritative micturition complaints, pelvic pain, involuntary urine loss or urinary retention are sometimes difficult to treat. The advent of direct sacral nerve stimulation offers a therapeutic alternative if conservative measures fail and surgery is considered. This paper reviews therapeutic neuromodulation by implant for treating lower urinary tract symptoms and dysfunction. The international literature is reviewed on topics such as the physiological basis of neuromodulation, techniques of acute testing and chronic implantation, and clinical results. Future developments and ways for possible improvement are discussed. The mode of action of neuromodulation is probably through restoring the correct balance between excitatory and inhibitory impulses from and to the pelvic organs at a sacral and supra-sacral level. Depending on the predefined success criteria, average success rates of definitive implants vary from 50 to 70%. From the data it seems that patients with urge incontinence and urinary retention are the best candidates for neuromodulation. In the literature the lack of standardisation of selection criteria, stimulation parameters and definitions of success is striking. Neuromodulation by implant is a useful therapeutic alternative. It should at least be considered in patients with therapy-resistant urge incontinence and urinary retention before proceeding to surgery. Issues such as underlying physiology, methodological standardisation, technical improvements, and patient selection must be addressed in future research.

Successful treatment of paraneoplastic hypercalcemia in a patient with giant condyloma acuminatum: a case report.

PubMed

Linnemann, Thomas; Müller, Frauke; Löhnert, Mathias; Hirnle, Peter; Görner, Martin

2013-11-07

While paraneoplastic syndromes in patients with malignant and metastasizing tumors are common, they are rarely associated with skin tumors showing predominantly local growth patterns. This case report relates to a patient with giant condyloma acuminatum, also called Buschke-Löwenstein tumor, with paraneoplastic hypercalcemia, who was successfully treated with conservative treatment. The patient in question is a 48-year-old German man with a giant periscrotal tumor. Before and during the therapy, two episodes of symptomatic hypercalcemia occurred, which were successfully treated by bisphosphonates, intravenous fluids and diuretics. No evidence of lytic bone affection was found. Paraneoplastic hypercalcemia may occur in patients who have a Buschke-Löwenstein tumor. For patients, where surgery is not an option, established medical therapies like bisphosphonates may be useful in addition to diuretics and infusions.

Hyponatremia due to Secondary Adrenal Insufficiency Successfully Treated by Dexamethasone with Sodium Chloride.

PubMed

Kazama, Itsuro; Tamada, Tsutomu; Nakajima, Toshiyuki

2015-08-28

Patients who were surgically treated for Cushing's syndrome postoperatively surrender to "primary" adrenal insufficiency. However, the preoperative over-secretion of cortisol or the postoperative administration of excessive glucocorticoids can cause "secondary" adrenal insufficiency, in which the prevalence of hyponatremia is usually lower than that of primary adrenal insufficiency. A 60-year-old woman with a past medical history of Cushing's syndrome developed hyponatremia with symptoms of acute glucocorticoid deficiency, such as prolonged general fatigue and anorexia, after upper respiratory tract infection. A decrease in the serum cortisol level and the lack of increase in the ACTH level, despite the increased demand for cortisol, enabled a diagnosis of "secondary" adrenal insufficiency. Although the initial fluid replacement therapy was not effective, co-administration of dexamethasone and sodium chloride quickly resolved her symptoms and ameliorated the refractory hyponatremia. In this case, the hypothalamic-pituitary axis of the patient was thought to have become suppressed long after the surgical treatment for Cushing's syndrome. This case suggested a mechanism of refractory hyponatremia caused by secondary adrenal insufficiency, for which the administration of dexamethasone and sodium chloride exerted additional therapeutic efficacy.

Retrograde Intrarenal Surgery versus Percutaneous Lithotripsy to Treat Renal Stones 2-3 cm in Diameter

PubMed Central

Zengin, Kursad; Tanik, Serhat; Sener, Nevzat Can; Albayrak, Sebahattin; Tuygun, Can; Bakirtas, Hasan; Imamoglu, M. Abdurrahim; Gurdal, Mesut

2015-01-01

Objective. Retrograde intrarenal surgery (RIRS) performed using a flexible ureterorenoscope marked the beginning of a new era in urology. Today, even staghorn stones are successfully treated via RIRS. The recommended treatment for larger stones is percutaneous nephrolithotomy (PNL). However, the question of whether PNL or RIRS should be the first-line treatment option for larger stones remains controversial. In this study, we contribute to the debate by comparing the success and complication rates of PNL and RIRS that were used to treat renal pelvis stones 2-3 cm in diameter. Materials and Methods. The medical records of 154 patients (74 PNL, 80 RIRS) were retrospectively evaluated. PNL patients were placed in Group 1 and RIRS patients in Group 2. Results. The complete stone-free rates were 95.5% in the PNL group and 80.6% in the RIRS group 1 month postoperatively (P = 0.061). The respective complication rates (evaluated using the Clavien system) were 13.5% and 8.8% (P = 0.520). Conclusions. RIRS affords a comparable success rate, causes fewer complications than PNL, and seems to be a promising alternative to PNL when larger stones are to be treated. Prospective randomized controlled trials are needed to confirm these findings. PMID:25821828

Case presentation: a novel way of treating acute cauliflower ear in a professional rugby player.

PubMed

Macdonald, D J M; Calder, N; Perrett, G; McGuiness, R G

2005-06-01

Acute auricular haematoma is a common problem in rugby players and can be difficult to treat due to re-accumulation of the fluid and can subsequently cause the unsightly cauliflower ear. We present a case of auricular haematoma affecting the central part of the pinna in a professional rugby player. This was treated successfully by aspiration and the use of silicone splints which allowed the player to continue training and competing.

The simultaneous occurrence of multiple myeloma and JAK2 positive myeloproliferative neoplasms - Report on two cases

PubMed Central

Badelita, S; Dobrea, C; Colita, A; Dogaru, M; Dragomir, M; Jardan, C; Coriu, D

2015-01-01

Multiple myeloma and JAK2 positive chronic myeloproliferative neoplasms are hematologic malignancies with a completely different cellular origin. Two cases of simultaneous occurrence of multiple myeloma, one with primary myelofibrosis and another one with essential thrombocythemia are reported in this article. In such cases, an accurate diagnosis requires a molecular testing, including gene sequencing and differential diagnosis of pancytosis associated with splenic amyloidosis. In general, in such cases, of two coexisting malignant hematologic diseases, the treatment of the most aggressive one is recommended. For our two cases, it was decided to start a Velcade based therapy. The main concern was the medullar toxicity, especially when a multiple myeloma was associated with a primary myelofibrosis. Abbreviations:JAK2 = Janus kinase 2 gene, PMF = primary myelofibrosis, MPNs = myeloproliferative neoplasms, ET = essential thrombocythemia, PV = polycythemia vera, MM = multiple myeloma, WBC = white blood cells, Hb = haemoglobin, Ht = haematocrit, Plt = platelets, BMB = bone marrow biopsy, CBC = blood cell count, CT = computerized tomography, LAP = leukocyte alkaline phosphatase, MGUS = monoclonal gammopathy of undetermined significance. PMID:25914740

Screening for MPL mutations in essential thrombocythemia and primary myelofibrosis: normal Mpl expression and absence of constitutive STAT3 and STAT5 activation in MPLW515L-positive platelets.

PubMed

Glembotsky, Ana C; Korin, Laura; Lev, Paola R; Chazarreta, Carlos D; Marta, Rosana F; Molinas, Felisa C; Heller, Paula G

2010-05-01

To evaluate the frequency of MPL W515L, W515K and S505N mutations in essential thrombocythemia (ET) and primary myelofibrosis (PMF) and to determine whether MPLW515L leads to impaired Mpl expression, constitutive STAT3 and STAT5 activation and enhanced response to thrombopoietin (TPO). Mutation detection was performed by allele-specific PCR and sequencing. Platelet Mpl expression was evaluated by flow cytometry, immunoblotting and real-time RT-PCR. Activation of STAT3 and STAT5 before and after stimulation with increasing concentrations of TPO was studied by immunoblotting. Plasma TPO was measured by ELISA. MPLW515L was detected in 1 of 100 patients with ET and 1 of 11 with PMF. Platelets from the PMF patient showed 100% mutant allele, which was <50% in platelets from the ET patient, who also showed the mutation in granulocytes, monocytes and B cells. Mpl surface and total protein expression were normal, and TPO levels were mildly increased in the MPLW515L-positive ET patient, while MPL transcripts did not differ from controls in both MPLW515L-positive patients. Constitutive STAT3 and STAT5 phosphorylation was absent and dose response to TPO-induced phosphorylation was not enhanced. The low frequency of MPL mutations in this cohort is in agreement with previous studies. The finding of normal Mpl levels in MPLW515L-positive platelets indicates this mutation does not lead to dysregulated Mpl expression, as frequently shown for myeloproliferative neoplasms. The lack of spontaneous STAT3 and STAT5 activation and the normal response to TPO is unexpected as MPLW515L leads to constitutive receptor activation and hypersensitivity to TPO in experimental models.

Tako-Tsubo cardiomyopathy presenting with cardiogenic shock successfully treated with milrinone: a case report.

PubMed

Doyen, Denis; Dellamonica, Jean; Moceri, Pamela; Moschietto, Sébastien; Hyvernat, Hervé; Ferrari, Emile; Bernardin, Gilles

2014-01-01

We report the case of a middle age patient presenting with Tako-Tsubo cardiomyopathy (TTC) complicated by cardiogenic shock that was successfully handled with milrinone. A 64-year old man presented with cardiogenic shock after benzodiazepine and alcohol intoxication. A slight elevation of troponin and typical left ventricular ballooning without coronary lesions suggested TTC. Within a few hours milrinone infusion normalized the cardiac index. TTC is responsible for severe transient left ventricular dysfunction occurring after physical or psychological stress. The major pathophysiological mechanism involved is disproportionate catecholamine secretion, which may stun the myocardium. We considered if treatment of this unique physiopathology with catecholamines could be dangerous in these patients and if alternative inotropes such as milrinone should be preferred. Copyright © 2014 The Authors. Published by Mosby, Inc. All rights reserved.

Where to Turn for Second-Line Cytoreduction After Hydroxyurea in Polycythemia Vera?

PubMed

Nazha, Aziz; Gerds, Aaron T

2016-04-01

The goals of therapy in patients with polycythemia vera (PV) are to improve disease-related symptoms, prevent the incidence or recurrence of thrombosis, and possibly delay or prevent the transformation into myelofibrosis or acute myeloid leukemia (AML). Cytoreductive therapies have been used in older patients and those with a history of thrombosis to achieve these goals. Hydroxyurea (HU) remains the first-line cytoreductive choice; however, up to one in four patients treated with HU over time will develop resistance or intolerance to HU. More importantly, patients who fail HU have a 5.6-fold increase in mortality and a 6.8-fold increase risk of transformation to myelofibrosis or AML; therefore, alternative therapies are needed for these patients. Interferon-α has been used in PV and has shown significant activity in achieving hematologic responses and decreasing JAK2 V617F mutation allele burden. JAK inhibition has also been investigated and recently garnered regulatory approval for this indication. In this review, we will discuss the current treatment options that are available for patients after HU and the novel therapies that are currently under investigation. The outcomes of PV patients who fail or who are intolerant of hydroxyurea are poor. Although pegylated interferon can be considered in younger patients, currently, ruxolitinib is the only U.S. Food and Drug Administration-approved agent in this setting, representing a viable option, leading to hematocrit control and a reduction in spleen size and constitutional symptoms. Although a small number of patients will achieve a molecular response with continuous treatment, the implications of such response on the clinical outcomes are still unknown. Patients whose disease is not adequately controlled with ruxolitinib, or who lose their response, can be treated with low-dose busulfan or pipobroman; however, they should be encouraged to participate in trials with novel therapies. ©AlphaMed Press.

Success of Dental Treatments under Behavior Management, Sedation and General Anesthesia.

PubMed

Blumer, Sigalit; Costa, Liora; Peretz, Benjamin

To present comparative study aims to assist the practitioner to choose between behavior modification (BM) techniques, pharmacologic sedation (N 2 O-O 2 alone or combined with midazolam 0.5 mg/ kg) or routine general anesthesia (GA) for the most successful approach in enabling pediatric dental care. Dental records of 56 children treated in a university dental clinic between 2006-2016 were reviewed, and data on age, gender, required treatment (amalgam restorations, composite restorations, pulpotomy, and stainless steel crowns [SSC]), treatment approaches and therapeutic success at final follow-up were retrieved. Treatment under GA had the best success rates compared to both BM and pharmacologic sedation. N 2 O-O 2 alone had a 6.1-fold greater risk of failure compared to N 2 O-O 2 +midazolam (p- <0.008). Amalgam restorations had a 2.61-fold greater risk of failure than SSC (p- <0.008). The GA mode yielded significantly greater success than the N 2 O-O 2 mode alone. There were no significant differences in success rates between GA and combined midazolam 0.5 mg/kg+N 2 O-O 2 . When choosing restoration material, it is important to remember the high success rate of SSC compared to amalgam restoration.

Halo vest treatment of cervical spine injuries: a success and survivorship analysis.

PubMed

Bransford, Richard J; Stevens, David W; Uyeji, Staci; Bellabarba, Carlo; Chapman, Jens R

2009-07-01

A retrospective study of a consecutive series of traumatic cervical spine injuries treated with halo vest immobilization (HVI) over an 8-year period at a level 1 trauma center. To assess survivorship, success, and causes of failure of HVI in the management of cervical spine injuries. The use of HVI has been increasingly questioned as an immobilization technique in cervical trauma due to reports of high complication rates and unacceptable treatment results. It was our hypothesis that selective use of updated HVI could demonstrate higher clinical success rates and lower complication rates compared to several previous landmark studies. All patients with traumatic cervical spine injuries treated with HVI between 1998 and 2006 at a single level 1 trauma center were reviewed retrospectively. With Internal Review Board approval, the trauma, spine, and orthotics databases were reviewed for (1) injury type, (2) patient age, (3) complications and comorbidities, (4) survivorship of the device and (5) treatment outcome. Four hundred ninety traumatic cervical spine injuries in 342 patients were treated with HVI. Thirty-one (9%) patients were lost to follow-up. Average age was 41 years (2-94). HVI was used as definitive treatment in 288 (84%) patients and in conjunction with surgical intervention in 54 (16%) patients. One hundred thirteen (35%) complications occurred, the most common of which were pin site infections (39) and instability (38). Two hundred seven (74%) of the 289 halo survivors with appropriate follow-up completed the initially prescribed time period of HVI. Two hundred eight of 247 (85%) halos placed as stand-alone management achieved their intended goal. Treatment with HVI was successful in 85% of patients and 74% of survivors completed their intended treatment period. Complications, though common, were mostly not severe. HVI is still a reasonable treatment option in managing cervical spine injuries.

Candida periprosthetic joint infection: A rare and difficult-to-treat infection.

PubMed

Escolà-Vergé, Laura; Rodríguez-Pardo, Dolors; Lora-Tamayo, Jaime; Morata, Laura; Murillo, Oscar; Vilchez, Helem; Sorli, Luisa; Carrión, Laura Guío; Barbero, José Mª; Palomino-Nicás, Julián; Bahamonde, Alberto; Jover-Sáenz, Alfredo; Benito, Natividad; Escudero, Rosa; Sampedro, Marta Fernandez; Vidal, Rafael Pérez; Gómez, Lucía; Corona, Pablo S; Almirante, Benito; Ariza, Javier; Pigrau, Carles

2018-05-08

Candida periprosthetic joint infection (CPJI) is a rare, difficult-to-treat disease. The purpose of this study was to evaluate the clinical characteristics and outcomes of CPJI treated with various surgical and antifungal strategies. We conducted a multicenter retrospective study of all CPJI diagnosed between 2003 and 2015 in 16 Spanish hospitals. Forty-three patients included: median age, 75 years, and median Charlson Comorbidity Index score, 4. Thirty-four (79.1%) patients had ≥1 risk factor for Candida infection. Most common causative species were C. albicans and C. parapsilosis. Thirty-five patients were evaluable for outcome: overall, treatment succeeded in 17 (48.6%) and failed in 18 (51.4%). Success was 13/20 (67%) in patients with prosthesis removal and 4/15 (27%) with debridement and prosthesis retention (p = 0.041). All 3 patients who received an amphotericin B-impregnated cement spacer cured. In the prosthesis removal group, success was 5/6 (83%) with an antibiofilm regimen and 8/13 (62%) with azoles (p = 0.605). In the debridement and prosthesis retention group, success was 3/10 (30%) with azoles and 1/5 (20%) with antibiofilm agents. Therapeutic failure was due to relapse in 9 patients, need for suppressive treatment in 5, persistent infection in 2, and CPJI-related death in 2; overall attributable mortality was 6%. CPJI is usually a chronic disease in patients with comorbidities and risk factors for Candida infection. Treatment success is low, and prosthesis removal improves outcome. Although there is insufficient evidence that use of antifungals with antibiofilm activity has additional benefits, our experience indicates it may be recommendable. Copyright © 2018 Elsevier Ltd. All rights reserved.

Leiomyosarcoma of the stomach treated by endoscopic submucosal dissection.

PubMed

Sato, Takao; Akahoshi, Kazuya; Tomoeda, Naru; Kinoshita, Norikatsu; Kubokawa, Masaru; Yodoe, Kentaro; Hiraki, Yuka; Oya, Masafumi; Yamamoto, Hidetaka; Ihara, Eikichi

2018-03-02

There have been no reports of primary leiomyosarcoma of the stomach treated by endoscopic submucosal dissection (ESD). We report an extremely rare case of gastric leiomyosarcoma that was successfully treated by ESD. An asymptomatic 74-year-old female underwent esophagogastroduodenoscopy for screening in December 2013. A centrally depressed submucosal tumor 10 mm in diameter was detected at the posterior wall of the upper gastric body. Follow-up esophagogastroduodenoscopy conducted 5 months later showed that the tumor diameter had increased to 15 mm. Endoscopic ultrasound revealed a hypoechoic mass located in the second to the middle of the third layer. Endoscopic ultrasound-guided fine-needle aspiration demonstrated a myogenic tumor. The tumor was completely resected by ESD without complications. Immunohistopathological diagnosis of the resected specimen was gastric leiomyosarcoma derived from the muscularis mucosae, with negative lateral and vertical margins. No local recurrence or metastasis has been detected at 36 months after ESD. This is the first report of gastric leiomyosarcoma treated by ESD in the English language literature.

Cognitive success: instrumental justifications of normative systems of reasoning

PubMed Central

Schurz, Gerhard

2014-01-01

In the first part of the paper (sec. 1–4), I argue that Elqayam and Evan's (2011) distinction between normative and instrumental conceptions of cognitive rationality corresponds to deontological vs. teleological accounts in meta-ethics. I suggest that Elqayam and Evans' distinction be replaced by the distinction between a-priori intuition-based vs. a-posteriori success-based accounts of cognitive rationality. The value of cognitive success lies in its instrumental rationality for almost-all practical purposes. In the second part (sec. 5–7), I point out that the Elqayam and Evans's distinction between normative and instrumental rationality is coupled with a second distinction: between logically general vs. locally adaptive accounts of rationality. I argue that these are two independent distinctions that should be treated as independent dimensions. I also demonstrate that logically general systems of reasoning can be instrumentally justified. However, such systems can only be cognitively successful if they are paired with successful inductive reasoning, which is the area where the program of adaptive (ecological) rationality emerged, because there are no generally optimal inductive reasoning methods. I argue that the practical necessity of reasoning under changing environments constitutes a dilemma for ecological rationality, which I attempt to solve within a dual account of rationality. PMID:25071624

Cognitive success: instrumental justifications of normative systems of reasoning.

PubMed

Schurz, Gerhard

2014-01-01

In the first part of the paper (sec. 1-4), I argue that Elqayam and Evan's (2011) distinction between normative and instrumental conceptions of cognitive rationality corresponds to deontological vs. teleological accounts in meta-ethics. I suggest that Elqayam and Evans' distinction be replaced by the distinction between a-priori intuition-based vs. a-posteriori success-based accounts of cognitive rationality. The value of cognitive success lies in its instrumental rationality for almost-all practical purposes. In the second part (sec. 5-7), I point out that the Elqayam and Evans's distinction between normative and instrumental rationality is coupled with a second distinction: between logically general vs. locally adaptive accounts of rationality. I argue that these are two independent distinctions that should be treated as independent dimensions. I also demonstrate that logically general systems of reasoning can be instrumentally justified. However, such systems can only be cognitively successful if they are paired with successful inductive reasoning, which is the area where the program of adaptive (ecological) rationality emerged, because there are no generally optimal inductive reasoning methods. I argue that the practical necessity of reasoning under changing environments constitutes a dilemma for ecological rationality, which I attempt to solve within a dual account of rationality.

Chylothorax Associated with Substernal Goiter in Graves' Disease Treated with Radioactive Iodine.

PubMed

Young Oh, Seo; Hyun Kim, Bo; Young Kim, Do; Min Lee, Kyu; Jin Lee, Min; Su Kim, Sung; Ho Kim, Jong; Kyung Jeon, Yun; Soo Kim, Sang; Ki Kim, Yong; Joo Kim, In

2017-04-01

We present a rare case of chylothorax associated with an intrathoracic goiter in Graves' disease that was treated with radioactive iodine. A 23-year-old woman with Graves' disease was referred to our clinic with a pleural effusion, dyspnea, characteristic bilateral proptosis, and a diffuse goiter. The pleural fluid biochemistry was consistent with chylothorax. However, the chylothorax did not decrease with conservative therapy. Therefore, RAI was administered. Subsequently, the chylothorax and goiter improved more quickly than expected. This case illustrates that chylothorax associated with a substernal goiter in Graves' disease can be treated successfully with radioactive iodine instead of surgery.

Post-traumatic hepatic artery pseudoaneurysm treated with endovascular embolization and thrombin injection.

PubMed

Francisco, Lloret Estañ; Asunción, López Conesa; Antonio, Capel Alemán; Ricardo, Robles Campos; Manuel, Reus Pintado; Caridad, Marín Hernández

2010-02-27

Post-traumatic hepatic artery pseudoaneurysm is uncommon, appearing in approximately 1% of hepatic trauma cases. Most are extrahepatic (80%) and have a late onset. Although they are usually asymptomatic, they should always be treated becasue of the high risk of complications, especially breakage. Currently the treatment of choice is endovascular embolization with coils or the exclusion of the pseudoaneurysm using other intravascular devices. Recently there have been accounts of a treatment that combines embolization with coils and image-guided percutaneous human thrombin injection. We present a case of post-traumatic hepatic artery pseudoaneurysm that was successfully treated using this combined technique.

Post-traumatic hepatic artery pseudoaneurysm treated with endovascular embolization and thrombin injection

PubMed Central

Francisco, Lloret Estañ; Asunción, López Conesa; Antonio, Capel Alemán; Ricardo, Robles Campos; Manuel, Reus Pintado; Caridad, Marín Hernández

2010-01-01

Post-traumatic hepatic artery pseudoaneurysm is uncommon, appearing in approximately 1% of hepatic trauma cases. Most are extrahepatic (80%) and have a late onset. Although they are usually asymptomatic, they should always be treated becasue of the high risk of complications, especially breakage. Currently the treatment of choice is endovascular embolization with coils or the exclusion of the pseudoaneurysm using other intravascular devices. Recently there have been accounts of a treatment that combines embolization with coils and image-guided percutaneous human thrombin injection. We present a case of post-traumatic hepatic artery pseudoaneurysm that was successfully treated using this combined technique. PMID:21160978

Treating Refractory Cardiogenic Shock With the TandemHeart and Impella Devices: A Single Center Experience

PubMed Central

Schwartz, Bryan G.; Ludeman, Daniel J.; Mayeda, Guy S.; Kloner, Robert A.; Economides, Christina; Burstein, Steven

2012-01-01

Background Patients with cardiogenic shock (CS) are routinely treated with intra-aortic balloon pumps (IABPs). The utility of 2 new percutaneous left ventricular assist devices (PLVADs), the Impella and TandemHeart, is unknown. The objective of this study was to describe the use of PLVADs for patients with CS at our institution. Methods All cases involving PLVADs in patients with CS between between January 1, 2008 and June 30, 2010 at a private, tertiary referral hospital were reviewed retrospectively. Results All 76 cases were identified (50 IABP only, 7 Impella, 19 TandemHeart). Most Impella (5/7) and TandemHeart (10/19) patients were initially treated with an IABP before "upgrading" for increased hemodynamic support. All 76 devices (100%) were initiated successfully. Percutaneous revascularization was attempted in 63 patients with angiographic success in 57 (90%). The incidences of major complications were similar between groups, except bleeding occurred less frequently with the IABP. Mean ejection fraction on presentation was 30.4±16.5% and increased by a mean of 6.6±11.4% (P < 0.001). With the institutional approach of treating patients with CS initially with vasopressors and IABPs, then upgrading to an Impella or TandemHeart device for patients refractory to IABP therapy, the overall mortality rate was 40%. Conclusion The Impella and TandemHeart devices can be initiated successfully in patients with CS, are associated with high rates of angiographic success during high risk percutaneous interventions and may benefit the myocardium during myocardial infarction. Randomized trials are warranted investigating use of the Impella and TandemHeart devices in patients with CS and in patients refractory to conventional IABP therapy. PMID:28348673

[MRI evaluation of placenta accreta treated by embolization. Apropos of a case. Review of the literature].

PubMed

Lemercier, E; Genevois, A; Descargue, G; Clavier, E; Benozio, M

1999-04-01

Placenta accreta results from an abnormal attachment of the placenta to the uterine myometrium. The reported incidence in literature is variable, with an average of 1/7000 pregnancies. This condition is associated with a significant risk of bleeding at the time of delivery, usually requiring hysterectomy. Sonography associated with color Doppler is useful for diagnosis, but MRI can be used successfully to evaluate the degree of placental tissue invading into the myometrium, the serosa, and for follow-up after conservative management. To our knowledge, only two cases of placenta accreta evaluated with MR and six cases of placenta accreta treated by embolization have been reported in the literature. The authors report one case of placenta accreta treated successfully by embolization, and followed-up by MRI.

Percutaneous implantation of a unibody endograft to treat non-aneurysmal aortoiliac disease.

PubMed

Cury, Marcus Vinícius Martins; Rodrigues, Thiago Osawa; Issa, Antônio Augusto Tadeu

2017-03-01

This case-series report describes the use of an Endologix AFX ® stent graft for the treatment of non-aneurysmal aortic disease. Over a period of 26 months, this device was successfully implanted in six patients with aortic bifurcation < 15 mm to treat critical limb ischemia secondary to dissection, ulcer, or coarctation.

Safe and successful endoscopic initial treatment and long-term eradication of gastric varices by endoscopic ultrasound-guided Histoacryl (N-butyl-2-cyanoacrylate) injection.

PubMed

Gubler, Christoph; Bauerfeind, Peter

2014-09-01

Optimal endoscopic treatment of gastric varices is still not standardized nowadays. Actively bleeding varices may prohibit a successful endoscopic injection therapy of Histoacryl® (N-butyl-2-cyanoacrylate). Since 2006, we have treated gastric varices by standardized endoscopic ultrasound (EUS) guided Histoacryl injection therapy without severe adverse events. We present a large single-center cohort over 7 years with a standardized EUS-guided sclerotherapy of all patients with gastric varices. Application was controlled by fluoroscopy to immediately detect any glue embolization. Only perforating veins located within the gastric wall were treated. In the follow up, we repeated this treatment until varices were eradicated. Utmost patients (36 of 40) were treated during or within 24 h of active bleeding. About 32.5% of patients were treated while visible bleeding. Histoacryl injection was always technically successful and only two patients suffered a minor complication. Acute bleeding was stopped in all patients. About 15% (6 of 40) of patients needed an alternative rescue treatment in the longer course. Three patients got a transjugular portosystemic shunt and another three underwent an orthotopic liver transplantation. Mean long-term survival of 60 months was excellent. Active bleeding of gastric varices can be treated successfully without the necessity of gastric rinsing with EUS-guided injection of Histoacryl.

Successful treatment of paraneoplastic hypercalcemia in a patient with giant condyloma acuminatum: a case report

PubMed Central

2013-01-01

Introduction While paraneoplastic syndromes in patients with malignant and metastasizing tumors are common, they are rarely associated with skin tumors showing predominantly local growth patterns. This case report relates to a patient with giant condyloma acuminatum, also called Buschke-Löwenstein tumor, with paraneoplastic hypercalcemia, who was successfully treated with conservative treatment. Case presentation The patient in question is a 48-year-old German man with a giant periscrotal tumor. Before and during the therapy, two episodes of symptomatic hypercalcemia occurred, which were successfully treated by bisphosphonates, intravenous fluids and diuretics. No evidence of lytic bone affection was found. Conclusions Paraneoplastic hypercalcemia may occur in patients who have a Buschke-Löwenstein tumor. For patients, where surgery is not an option, established medical therapies like bisphosphonates may be useful in addition to diuretics and infusions. PMID:24200238

Intravenous Lidocaine Infusion to Treat Chemotherapy-Induced Peripheral Neuropathy.

PubMed

Papapetrou, Peter; Kumar, Aashish J; Muppuri, Rudram; Chakrabortty, Shushovan

2015-11-01

Chemotherapy-induced peripheral neuropathy is a debilitating side effect of chemotherapy, which manifests as paresthesias, dysesthesias, and numbness in the hands and feet. Numerous chemoprotective agents and treatments have been used with limited success to treat chemotherapy-induced peripheral neuropathy. We report a case in which a patient presenting with chemotherapy-induced peripheral neuropathy received an IV lidocaine infusion over the course of 60 minutes with complete symptomatic pain relief for a prolonged period of 2 weeks.

[Successful treatment of Candida meningitis with miconazole].

PubMed

Fukui, S; Tabata, H; Hayashi, H; Matsushima, Y

1990-09-01

This paper presents a case of successful treatment of candida meningitis with miconazole. A 55-year-old woman was admitted due to high fever, vomiting and urinary incontinence on November 11, 1986. Four months prior to this episode, she had been treated for a ruptured aneurysm with neck-clipping and V-P shunt for NPH. Candida albicans was cultured from her CSF. The shunt system was immediately removed and an Ommaya's reservoir was installed for external drainage and intrathecal administrations. Combination therapy (amphotericin B and flucytosine) was initiated. However, it was discontinued after ten days because of high fever and chills after intrathecal injection of amphotericin B. Treatment with miconazole intrathecally (10-90 mg/week, total 565 mg) and intravenously (200-1200 mg/day, total 70.4 g) was begun on November 23. Clinical and CSF findings were improved soon. No side effect of miconazole was observed. After V-P shunt revision, she was discharged without neurological deficit on March 12, 1987. Reports of mycosis in central nervous system are recently increasing, especially for candidosis. Cryptococcosis is noted frequently as an opportunistic infection of AIDS. The administration of amphotericin B and flucytosine has been the main therapy for mycotic meningitis. Unfortunately, however, Amphotericin B has many toxic effects, including renal dysfunction, and flucytosine can induce the emergent resistance. Miconazole has been used to successfully treat cryptococcosis, aspergillosis or coccidiosis, and was effective in our case of candida meningitis. Few side effects have been reported with its use. The intrathecal injection of miconazole is recommended for meningitis, because the drug is taken up minimally into CSF space after intravenous administration.(ABSTRACT TRUNCATED AT 250 WORDS)

Recurrent Hemarthrosis due to Iatrogenic AVF Treated With Onyx Embolization.

PubMed

Koleilat, Issam; Phair, John

2017-07-01

A 78-year-old gentleman presented with recurrent symptomatic hemarthrosis after total knee arthroplasty. His workup revealed an iatrogenic arteriovenous fistula (iAVF). The iAVF was embolized with the Onyx Liquid Embolization System with resolution of his symptoms up to 10 months of follow-up. This is the first description to our knowledge of an iatrogenic hemarthrosis after total knee arthroplasty successfully treated with Onyx solution embolization.

Successful treatment of eosinophilic pustular folliculitis with topical tacrolimus 0.1 percent ointment.

PubMed

Ng, Shanna Shan-Yi; Tay, Yong-Kwang

2012-02-15

Classic eosinophilic pustular folliculitis (EPF), otherwise known as Ofugi disease, is a rare condition commonly treated with topical glucocorticosteroids. If this fails, oral indomethacin is frequently the next line. Because the condition is recurrent, the use of long term steroids may cause side effects such as skin atrophy, hypertrichosis, and dyspigmentation. Topical tacrolimus is an immunosuppressant that is generally used as a steroid-sparing agent in atopic dermatitis. We report a case of classic EPF, which was recurrent over 5 years that had failed topical glucocorticosteroids but was successfully treated with topical tacrolimus 0.1 percent ointment.

A Phase Ib/II Dose-finding Study to Assess the Safety and Efficacy of LDE225 + INC424 in Patients With MF

ClinicalTrials.gov

2017-07-24

Primary Myelofibrosis; Thrombocythemia, Essential; Thrombocytosis; Myeloproliferative Disorders; Bone Marrow Diseases; Hematologic Diseases; Blood Coagulation Disorders; Blood Platelet Disorders; Hemorrhagic Disorders

Successful embolization of iatrogenic ruptured coronary artery using Onyx: a new technique.

PubMed

Asouhidou, I; Katsaridis, V

2014-12-01

Iatrogenic perforation of coronary artery is rare during percutaneous coronary intervention (PCI); however the complications are life-threatening. Patients in this clinical setting may be treated either by stent placement, closure of the perforation with fibrin glue or coils, or with emergency bypass surgery. Onyx, a new material that has been used successfully in cerebral arteries, represents a new and safe alternative. The advantage of Onyx is that it is easily injected through a microcatheter and it allows for a longer injection time having also the ability to reach difficult anatomical locations. We present the first case of successful embolization of a right coronary artery perforation during coronary angiography using Onyx.

Factors Associated with HIV Status Disclosure in HIV-Infected Sub-Saharan Migrants Living in France and Successfully Treated with Antiretroviral Therapy: Results from the ANRS-VIHVO Study.

PubMed

Kankou, J M; Bouchaud, O; Lele, N; Bourgeois, D; Spire, B; Carrieri, M P; Abgrall, S

2017-08-01

To estimate rates and identify correlates of HIV disclosure in migrants from sub-Saharan Africa (SSA) successfully treated, a sub-analysis was conducted in HIV-1 native SSA migrants, living in France with undetectable viral load on antiretroviral, included in the VIHVO adherence study. Logistic regression models assessed factors associated with HIV disclosure. Among 246 individuals (40 % male, median age 41), 79 % of those in a steady heterosexual partnership (n = 167) had disclosed their status to their partner, 55 % of the total 246 to a relative, and 33 % to (an)other person(s). Disclosure to one's steady partner was associated with a follow-up duration since HIV diagnosis of more than 5 years, a higher literacy level, a better social context and marital status. Women were more likely to disclose their HIV status to relatives. Interventions targeting this population should be provided to improve disclosure which in turn ensures better social support, testing of the partner and lower rates of undiagnosed HIV.

A Simple Hypnotic Approach to Treat Test Anxiety in Medical Students and Residents.

ERIC Educational Resources Information Center

Hebert, Stephen W.

1984-01-01

A simple hypnotic procedure to treat test anxiety is described that was used successfully with medical students and residents at the Wake Forest University Medical Center. A light trace is obtained and then the student is told to take such a hypnotic "journey" the evening prior to the test. (MLW)

College Success Courses: Success for All

ERIC Educational Resources Information Center

Coleman, Sandra Lee; Skidmore, Susan Troncoso; Weller, Carol Thornton

2018-01-01

College success courses (CSCs), or orientation courses, are offered by community colleges and universities to facilitate the success of first-time-in-college students. Primarily, these courses are designed to address students' nonacademic deficiencies, such as weak study habits and poor organizational skills, and to familiarize students with…

Invasive Fire Ants Reduce Reproductive Success and Alter the Reproductive Strategies of a Native Vertebrate Insectivore

PubMed Central

Ligon, Russell A.; Siefferman, Lynn; Hill, Geoffrey E.

2011-01-01

Background Introduced organisms can alter ecosystems by disrupting natural ecological relationships. For example, red imported fire ants (Solenopsis invicta) have disrupted native arthropod communities throughout much of their introduced range. By competing for many of the same food resources as insectivorous vertebrates, fire ants also have the potential to disrupt vertebrate communities. Methodology/Principal Findings To explore the effects of fire ants on a native insectivorous vertebrate, we compared the reproductive success and strategies of eastern bluebirds (Sialia sialis) inhabiting territories with different abundances of fire ants. We also created experimental dyads of adjacent territories comprised of one territory with artificially reduced fire ant abundance (treated) and one territory that was unmanipulated (control). We found that more bluebird young fledged from treated territories than from adjacent control territories. Fire ant abundance also explained significant variation in two measures of reproductive success across the study population: number of fledglings and hatching success of second clutches. Furthermore, the likelihood of bluebird parents re-nesting in the same territory was negatively influenced by the abundance of foraging fire ants, and parents nesting in territories with experimentally reduced abundances of fire ants produced male-biased broods relative to pairs in adjacent control territories. Conclusions/Significance Introduced fire ants altered both the reproductive success (number of fledglings, hatching success) and strategies (decision to renest, offspring sex-ratio) of eastern bluebirds. These results illustrate the negative effects that invasive species can have on native biota, including species from taxonomically distant groups. PMID:21799904

Spontaneous Massive Hemothorax in a Patient with Neurofibromatosis Type 1 with Successful Transarterial Embolization

PubMed Central

Rookkapan, Sorracha; Tanutit, Pramot; Pakdeejit, Songklod; Songjamrat, Apiradee; Sungsiri, Jitpreedee

2013-01-01

Vascular involvement in neurofibromatosis type 1 is rare but has the potential to be fatal. We report a case of a patient with spontaneous rupture of a left intercostal artery aneurysm, which presented as a massive left hemothorax and was successfully treated by transarterial coil embolization. PMID:23323035

Considerations in treating physically active older adults and aging athletes.

PubMed

Langer, Paul R

2015-04-01

Life spans are increasing and research is showing more and more how important exercise is to successful aging. Medical practitioners need to appreciate the physiologic and physical changes that occur with age, as well as the significant benefits of physical activity, so they not only can properly treat their older patients but also so they can promote the benefits of exercise to their sedentary older patients. Copyright © 2015 Elsevier Inc. All rights reserved.

Direct restoration of endodontically treated maxillary central incisors: post or no post at all?

PubMed

von Stein-Lausnitz, Manja; Bruhnke, M; Rosentritt, M; Sterzenbach, G; Bitter, K; Frankenberger, R; Naumann, M

2018-04-30

The aim of this ex-vivo study was to evaluate the impact of cavity size and glass-fiber post (GFP) placement on the load capability of endodontically treated maxillary incisors directly restored with resin composite. Ninety-six extracted human maxillary central incisors were endodontically treated and distributed to four groups (n = 24): access cavity (A), access cavity and uni-proximal class III cavity (U), access cavity and bi-proximal class III cavity (B), and decoronated tooth (D). Specimens were restored with resin composite, and 12 specimen of each group received an adhesively placed glass-fiber post (P). Prior to linear loading, specimens were exposed to thermo-mechanical loading (TCML). Statistical analysis was performed using log-rank test after TCML, Kruskall-Wallis and Mann-Whitney U test to compare load capabilities (F max) . Significantly more failures occurred in group D for specimens without GFP during TCML (p = 0.001). F max (mean (SD) in N was (A) 513 (124), (AP) 554 (201), (U) 438 (171), (UP) 537 (232) (B) 483 (219), (BP) 536 (281), D 143 (181), and DP 500 (331), and differed significantly among groups (p = 0.003). Pair-wise comparison revealed lower F max values for group D compared to all other groups (p < 0.034) except group DP. Endodontically treated maxillary central incisors with cavity sizes up to bi-proximal class III may be successfully directly restored with resin composite. Post placement shows no additional effect except for decoronated endodontically treated incisors. Endodontically treated incisors with access cavities to class III cavities can be successfully restored with resin composite. Post placement for decoronated ETT is recommended.

An Alternative Approach for Treating a Type Ia Endoleak after Conventional EVAR Using the Nellix Endovascular Aneurysm Sealing.

PubMed

Martinelli, Ombretta; Fresilli, Mauro; Irace, Luigi; Venosi, Salvatore; Jabbour, Jihad; Picone, Veronica; Maruca, Debora; Di Girolamo, Alessia; Gossetti, Bruno

2018-05-01

To report the use of a Nellix endovascular aneurysm sealing (EVAS) device, to successfully treat a type Ia endoleak (EL) after an endovascular aortic repair (EVAR). A 70-year-old man was diagnosed with a 90-mm aortic aneurysm, suspicious for being inflammatory. It was initially treated successfully, with a Medtronic Endurant (Medtronic, Minneapolis, MN, USA). Five years after the index endovascular repair, an asymptomatic type Ia EL was detected on duplex ultrasound and computed tomographic angiogram. Other endovascular solutions in the form of proximal cuff, chimney was considered difficult to execute due to challenges in planning, manipulation, and renal cannulation caused by the short proximal sealing zone above the existing stent graft and the constraints of the previous endograft. Thus, a relining of the previous endoprothesis was performed using the Nellix system (Endologix, Inc., Irvine, CA, USA). One-year follow-up imaging demonstrated successful resolution of the EL and persistent sealing of the Nellix device. Nellix EVAS system can be an alternative and safe option for relining a stent graft with a type Ia EL. Nellix platform can be added to the clinician's armamentarium for treating type Ia EL after conventional EVAR of infrarenal abdominal aortic aneurysm (AAA). Copyright © 2018 Elsevier Inc. All rights reserved.

Anti-Müllerian hormone as a predictor of polycystic ovary syndrome treated with clomiphene citrate.

PubMed

Hestiantoro, Andon; Negoro, Yuwono Sri; Afrita, Yohana; Wiweko, Budi; Sumapradja, Kanadi; Natadisastra, Muharam

2016-12-01

This study aimed to determine the threshold of anti-Müllerian hormone (AMH) as predictor of follicular growth failure in polycystic ovary syndrome (PCOS) patients treated with clomiphene citrate (CC). Fifty female subjects with PCOS were recruited and divided into two groups based on successful and unsuccessful follicular growth. Related variables such as age, infertility duration, cigarette smoking, use of Moslem hijab, sunlight exposure, fiber intake, body mass index, waist circumference, AMH level, 25-hydroxy vitamin D level, and growth of dominant follicles were obtained, assessed, and statistically analyzed. The AMH levels of patients with successful follicular growth were significantly lower ( p =0.001) than those with unsuccessful follicular growth (6.10±3.52 vs. 10.43±4.78 ng/mL). A higher volume of fiber intake was also observed in the successful follicular growth group compared to unsuccessful follicular growth group ( p =0.001). Our study found the probability of successful follicle growth was a function of AMH level and the amount of fiber intake, expressed as Y=-2.35+(-0.312×AMH level)+(0.464×fiber intake) (area under the curve, 0.88; 95% confidence interval, 0.79-0.98; p <0.001). The optimal threshold of AMH level in predicting the failure of follicle growth in patients with PCOS treated with CC was 8.58 ng/mL.

A Selective Mutism Arising from First Language Attrition, Successfully Treated with Paroxetine-CBT Combination Treatment.

PubMed

Serra, Agostino; Di Mauro, Paola; Andaloro, Claudio; Maiolino, Luigi; Pavone, Piero; Cocuzza, Salvatore

2015-10-01

After immersion in a foreign language, speakers often have difficulty retrieving native-language words and may experience a decrease in its proficiency, this phenomenon, in the non-pathological form, is known as first language attrition. Self-perception of this low native-language proficiency and apprehension occurring when speaking is expected and, may sometimes lead these people to a state of social anxiety and, in extreme forms, can involve the withholding of speech as a primitive tool for self-protection, linking them to selective mutism. We report an unusual case of selective mutism arising from first language attrition in an Italian girl after attending a two-year "German language school", who successfully responded to a paroxetine-cognitive behavioral treatment (CBT) combination treatment.

A Selective Mutism Arising from First Language Attrition, Successfully Treated with Paroxetine-CBT Combination Treatment

PubMed Central

Serra, Agostino; Di Mauro, Paola; Andaloro, Claudio; Maiolino, Luigi; Pavone, Piero

2015-01-01

After immersion in a foreign language, speakers often have difficulty retrieving native-language words and may experience a decrease in its proficiency, this phenomenon, in the non-pathological form, is known as first language attrition. Self-perception of this low native-language proficiency and apprehension occurring when speaking is expected and, may sometimes lead these people to a state of social anxiety and, in extreme forms, can involve the withholding of speech as a primitive tool for self-protection, linking them to selective mutism. We report an unusual case of selective mutism arising from first language attrition in an Italian girl after attending a two-year "German language school", who successfully responded to a paroxetine-cognitive behavioral treatment (CBT) combination treatment. PMID:26508972

Refractive eye surgery in treating functional amblyopia in children.

PubMed

Levenger, Samuel; Nemet, Pinhas; Hirsh, Ami; Kremer, Israel; Nemet, Arie

2006-01-01

While excimer laser refractive surgery is recommended and highly successful for correcting refractive errors in adults, its use in children has not been extensively exercised or studied. We report our experience treating children with amblyopia due to high anisometropia, high astigmatism, high myopia and with associated developmental delay. Review of patient records of our refractive clinic. A retrospective review was made of all 11 children with stable refractive errors who were unsuccessfully treated non-surgically and then underwent corneal refractive surgery and in one case, lenticular surgery. Seven had high myopic anisometropia, 2 had high astigmatism, and two had high myopia--one with Down's Syndrome and one with agenesis of the corpus callosum. The surgical refractive treatment eliminated or reduced the anisometropia, reduced the astigmatic error, improved vision and improved the daily function of the children with developmental delay. There were no complications or untoward results. Refractive surgery is safe and effective in treating children with high myopic anisometropia, high astigmatism, high myopia and developmental delay due to the resulting poor vision. Surgery can improve visual acuity in amblyopia not responding to routine treatment by correcting the refractive error and refractive aberrations.

Stress Fractures of Tibia Treated with Ilizarov External Fixator.

PubMed

Górski, Radosław; Żarek, Sławomir; Modzelewski, Piotr; Górski, Ryszard; Małdyk, Paweł

2016-08-30

Stress fractures are the result of cyclic loading of the bone, which gradually becomes damaged. Most often they are treated by rest or plaster cast and, in rare cases, by internal fixation. There is little published data on initial reposition followed by stabilization with the Ilizarov apparatus in such fractures. Six patients were treated with an external fixator according to the Ilizarov method for a stress fracture of the tibia between 2007 and 2015. Three patients were initially treated conservatively. Due to increasing tibial deformation, they were qualified for surgical treatment with external stabilization. In the other patients, surgery was the first-line treatment. All patients demonstrated risk factors for a stress fracture. After the surgery, they fully loaded the operated limb. No patient developed malunion, nonunion, infection or venous thrombosis. The average time from the first operation to the removal of the external fixator was 19 weeks. Radiographic and clinical outcomes were satisfactory in all patients. 1. The Ilizarov method allows for successful stabilization of stress fractures of the tibia. 2. It may be a good alternative to internal stabilization, especially in patients with multiple comorbidities which affect bone quality and may impair soft tissue healing.

Successful Treatment of Generalized Essential Telangiectasia With 6-Mercaptopurine.

PubMed

Glazer, Alex M; Sofen, Bryan D; Rigel, Darrell S; Shupack, Jerome L

2017-03-01

Generalized essential telangiectasia (GET) is a notoriously difficult to treat disorder with no current satisfactory treatments. This case and discussion report the use of 6-mercaptopurine (6-MP) as a successful treatment for GET. Moreover, we show that GET may represent a state of increased angiogenesis, a paradigm shift from the current understanding that these telangiectasias represent dilatations of only pre-existing vessels. This new view of GET may drive others to look at novel agents for treatment.

J Drugs Dermatol. 2017;16(3):280-282.

Polycythemia and Thrombocytosis.

PubMed

Parnes, Aric; Ravi, Arvind

2016-12-01

Myeloproliferative neoplasms (MPNs) are diseases of excess cell proliferation from bone marrow precursors. Two classic MPNs, polycythemia vera (PV) and essential thrombocytosis (ET), are conditions of excess proliferation of red blood cells and platelets, respectively. Although PV and ET involve different cells in the myeloid lineage, their clinical presentations have shared features, consistent with overlapping mutations in growth factor signaling. The management of both diseases involves minimizing the risk of thrombotic and hemorrhagic complications. Both PV and ET can progress to myelofibrosis or acute myeloid leukemia, portending a poor prognosis. MPNs can also present as primary myelofibrosis. Copyright © 2016 Elsevier Inc. All rights reserved.

Immediate stent recoil in an anastomotic vein graft lesion treated by cutting balloon angioplasty.

PubMed

Akkus, Nuri Ilker; Budeepalli, Jagan; Cilingiroglu, Mehmet

2013-11-01

Saphenous vein graft (SVG) anastomotic lesions can have significant fibromuscular hyperplasia and may be resistant to balloon angioplasty alone. Stents have been used successfully to treat these lesions. There are no reports of immediate stent recoil following such treatment in the literature. We describe immediate and persistent stent recoil in an anastomotic SVG lesion even after initial and post-deployment complete balloon dilatation of the stent and its successful treatment by cutting balloon angioplasty. Copyright © 2013 Sociedade Portuguesa de Cardiologia. Published by Elsevier España. All rights reserved.

Comparison of in vitro estrogenic activity and estrogen concentrations in source and treated waters from 25 U.S. drinking water treatment plants

EPA Science Inventory

In vitro bioassays have been successfully used to screen for estrogenic activity in wastewater and surface water, however, few have been applied to treated drinking water. Here, extracts of source and treated drinking water samples were assayed for estrogenic activity using T47D...

Tracing the origins of success: implications for successful aging.

PubMed

Peterson, Nora M; Martin, Peter

2015-02-01

This paper addresses the debate about the use of the term "successful aging" from a humanistic, rather than behavioral, perspective. It attempts to uncover what success, a term frequently associated with aging, is: how can it be defined and when did it first come into use? In this paper, we draw from a number of humanistic perspectives, including the historical and linguistic, in order to explore the evolution of the term "success." We believe that words and concepts have deep implications for how concepts (such as aging) are culturally and historically perceived. We take a comparative approach, turning to the etymological roots of this term in British, French, and German literature. According to the earliest entries of the term in the Oxford English Dictionary, events can have good or bad success. Another definition marks success as outcome oriented. Often used in the context of war, religion, and medicine, the neutral, but often negative, use of "success" in literature of the Renaissance demonstrates the tensions that surround the word, and suggests that success is something to be approached carefully. Ignoring the ambiguous origins of success erases the fact that aging in earlier centuries echoes much of the same ambivalence with which many people discuss it today. Attending to the origins of success can help gerontologists understand the humanistic tradition behind their inquiry into what successful aging means today. © The Author 2014. Published by Oxford University Press on behalf of The Gerontological Society of America. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

An Ice Block: A Novel Technique of Successful Prevention of Cement Leakage Using an Ice Ball

DOE Office of Scientific and Technical Information (OSTI.GOV)

Uri, Ishaq Fahmi, E-mail: uri.isaac@gmail.com; Garnon, Julien, E-mail: juliengarnon@gmail.com; Tsoumakidou, Georgia, E-mail: georgia.tsoumakidou@chru-strasbourg.fr

2015-04-15

We report three cases of painful bone metastases with extraosseous invasion treated with cementoplasty and cryoablation. Due to significant cortical loss in all cases, the ice ball was used simultaneously during cementoplasty to deter potential cement leakage. This was achieved by direct application of the ice ball against the cortical surface, resulting in adequate consolidation and successful containment of the cement within the treated bones. To the authors’ knowledge, this is the first report to describe such a combined technique.

Acute liver failure with thyrotoxicosis treated with liver transplantation.

PubMed

Cascino, Matthew D; McNabb, Brian; Gardner, David G; Woeber, Kenneth A; Fox, Alyson N; Wang, Bruce; Fix, Oren K

2013-01-01

We describe a young woman with previously undiagnosed thyrotoxicosis who presented with acute liver failure (ALF). We present a case report and review the relevant literature. An extensive evaluation excluded possible causes of ALF other than thyrotoxicosis. The management of thyrotoxicosis posed several unique challenges in the setting of ALF, particularly because we did not want to use potentially hepatotoxic thionamides. The patient was treated with prednisone and propranolol and was started on potassium iodide when she was listed for liver transplantation. She underwent an uncomplicated liver transplant and subsequent thyroidectomy and is doing well. This well-characterized case describes thyrotoxicosis as a possible cause of ALF after thoroughly excluding other possible causes and illustrates the challenges of simultaneously managing both disorders. To our knowledge, this is the first report of ALF possibly resulting from untreated thyrotoxicosis that was successfully treated with liver transplantation.

Buffered hydrochloric acid: a modern method of treating metabolic alkalosis.

PubMed

Finkle, D; Dean, R E

1981-03-01

Twenty-one patients with metabolic alkalosis were treated successfully with intravenous hydrochloric acid (HCl) buffered in an amino acid solution (TPN). No complications of HCl were seen. TPN was used to meet energy needs and provide a buffering effect through the interaction of HCl and amino acids. Buffered HCl therapy should be considered as the initial treatment in patients with metabolic alkalosis associated with congestive heart failure, renal failure, hepatic failure, cerebral edema, or refractory metabolic alkalosis.

Hepatitis C Care in the Department of Veterans Affairs: Building a Foundation for Success.

PubMed

Belperio, Pamela S; Chartier, Maggie; Gonzalez, Rachel I; Park, Angela M; Ross, David B; Morgan, Tim R; Backus, Lisa I

2018-06-01

The Department of Veterans Affairs (VA) has made significant progress in treating hepatitis C virus, experiencing more than a 75% reduction in veterans remaining to be treated since the availability of oral direct-acting antivirals. Hepatitis C Innovation Teams use lean process improvement and system redesign, resulting in practice models that address gaps in care. The key to success is creative improvements in veteran access to providers, including expanded use of nonphysician providers, video telehealth, and electronic technologies. Population health management tools monitor and identify trends in care, helping the VA tailor care and address barriers. Published by Elsevier Inc.

Successful medical management of intra-abdominal abscesses in 4 adult horses

PubMed Central

Berlin, Dalia; Kelmer, Gal; Steinman, Amir; Sutton, Gail A.

2013-01-01

Four adult horses with large intra-abdominal abscesses, suspected to be complications of strangles, were treated with systemic antibiotics alone and made a full recovery. The 100% survival rate is significantly better than other reported survival rates. The median duration of treatment (35 days) was shorter than in most previous reports. This study suggests that penicillin G can be used for successful treatment of strangles associated intra-abdominal abscesses in horses. PMID:23904639

Successful amplification of DNA aboard the International Space Station.

PubMed

Boguraev, Anna-Sophia; Christensen, Holly C; Bonneau, Ashley R; Pezza, John A; Nichols, Nicole M; Giraldez, Antonio J; Gray, Michelle M; Wagner, Brandon M; Aken, Jordan T; Foley, Kevin D; Copeland, D Scott; Kraves, Sebastian; Alvarez Saavedra, Ezequiel

2017-01-01

As the range and duration of human ventures into space increase, it becomes imperative that we understand the effects of the cosmic environment on astronaut health. Molecular technologies now widely used in research and medicine will need to become available in space to ensure appropriate care of astronauts. The polymerase chain reaction (PCR) is the gold standard for DNA analysis, yet its potential for use on-orbit remains under-explored. We describe DNA amplification aboard the International Space Station (ISS) through the use of a miniaturized miniPCR system. Target sequences in plasmid, zebrafish genomic DNA, and bisulfite-treated DNA were successfully amplified under a variety of conditions. Methylation-specific primers differentially amplified bisulfite-treated samples as would be expected under standard laboratory conditions. Our findings establish proof of concept for targeted detection of DNA sequences during spaceflight and lay a foundation for future uses ranging from environmental monitoring to on-orbit diagnostics.

Fludarabine Based Conditioning for Allogeneic Transplantation for Advanced Hematologic Malignancies

ClinicalTrials.gov

2017-10-25

Acute Myeloid Leukemia; Acute Leukemia; Chronic Myelogenous Leukemia; Malignant Lymphoma; Hodgkin's Disease; Multiple Myeloma; Lymphocytic Leukemia; Myeloproliferative Disorder; Polycythemia Vera; Myelofibrosis; Aplastic Anemia

Congenital Double Lip: A Rare Deformity Treated Surgically

PubMed Central

Aggarwal, Titiksha; Chawla, Kirti; Lamba, Arundeep Kaur; Faraz, Farukh; Tandon, Shruti

2016-01-01

Lip is an important aspect of facial features affecting ones personality. A deformity of the lip characterized by excessive tissue sagging below the usual giving it thicker wider appearance is referred to as double lip. It is a rare occurrence with a proposed male predilection. This article is a report of a 20 years old male with this deformity who presented with the complaint of difficult speech and poor aesthetics. There was no other history patient being systemically healthy. It was successively treated with a simple surgical technique without recurrence over a period of 12 months. PMID:27853696

Educational Attainment: Success to the Successful

ERIC Educational Resources Information Center

Anthony, Peter; Gould, David; Smith, Gina

2013-01-01

Systems archetypes are patterns of structure found in systems that are helpful in understanding some of the dynamics within them. The intent of this study was to examine educational attainment data using the success-to-the-successful archetype as a model to see if it helps to explain the inequality observed in the data. Data covering 1990 to 2009…

Successful prospective management of neonatal citrullinemia.

PubMed

Go, Hayato; Imamura, Takashi; Hashimoto, Koichi; Ogasawara, Kei; Sakamoto, Osamu; Takubo, Noriyuki; Momoi, Nobuo; Hosoya, Mitsuaki

2012-01-01

Classical citrullinemia generally involves hyperammonemic coma in the first few days of life and leads to neurological sequelae in survivors. We report a case of an elder sister who fell into a hyperammonemic coma on the fifth day after birth. She was successfully treated with intravenous benzoate and hemodialysis, and was subsequently diagnosed with citrullinemia on the basis of biochemical analysis. Two years later, a younger sister was born without prenatal diagnosis. We monitored plasma ammonia and citrulline levels after birth, and again diagnosed her with CTLN1 on the basis of biochemical and DNA analyses. There have been few reports of the prospective treatment of citrullinemia; however, our experience indicates the need for the prospective management and the rapid reduction of ammonia levels to avoid neonatal hyperammonemic coma and subsequent sequelae.

Tracing the Origins of Success: Implications for Successful Aging

PubMed Central

Peterson, Nora M.; Martin, Peter

2015-01-01

Purpose of the Study: This paper addresses the debate about the use of the term “successful aging” from a humanistic, rather than behavioral, perspective. It attempts to uncover what success, a term frequently associated with aging, is: how can it be defined and when did it first come into use? In this paper, we draw from a number of humanistic perspectives, including the historical and linguistic, in order to explore the evolution of the term “success.” We believe that words and concepts have deep implications for how concepts (such as aging) are culturally and historically perceived. Design and Methods: We take a comparative approach, turning to the etymological roots of this term in British, French, and German literature. According to the earliest entries of the term in the Oxford English Dictionary, events can have good or bad success. Another definition marks success as outcome oriented. Results: Often used in the context of war, religion, and medicine, the neutral, but often negative, use of “success” in literature of the Renaissance demonstrates the tensions that surround the word, and suggests that success is something to be approached carefully. Implications: Ignoring the ambiguous origins of success erases the fact that aging in earlier centuries echoes much of the same ambivalence with which many people discuss it today. Attending to the origins of success can help gerontologists understand the humanistic tradition behind their inquiry into what successful aging means today. PMID:24997595

The Unreasonable Success of Magnetosphere-Ionosphere Coupling Theory

NASA Astrophysics Data System (ADS)

Vasyliūnas, V. M.

2002-12-01

The description of plasma dynamics on the basis of self-consistent coupling between magnetosphere and ionosphere, as first systematized in the early 1970's, is arguably one of the most successful theories in magnetospheric physics. It accounts for the pattern of magnetospheric convection at auroral and low latitudes, the distribution of Birkeland currents, and the dependence on changing orientation of the interplanetary magnetic field. It can incorporate assumed effects, e.g. of particle sources or conductance variations, to almost any degree of complexity at moderate cost in additional computing effort (compare the levels of physics included in advanced versions of the Rice Convection Model and of global MHD simulations, respectively). Such success combined with relative simplicity, however, is possible only because the theory has limited itself in significant ways. It treats the system in effect as doubly two-dimensional: height-integrated ionosphere plus field-line-integrated magnetosphere, with the background magnetic field structure treated as known or derived from some empirical model. It assumes that the system is always in slowly evolving quasi-equilibrium and deals only with time scales long compared to wave propagation times. Hence the theory is not easily applied where genuine 3D aspects (e.g. height and field-line dependence), poorly known or variable magnetic fields (e.g. open field lines), or transient responses e.g. to rapid solar-wind changes are important, and it is intrinsically incapable of describing explosive non-equilibrium developments such as substorm onset. Possible extensions of the theory, comparison with numerical-simulation approaches, and implications for general space plasma physics (E-J vs. B-V) will be discussed.

Losartan enhances the success of myoblast transplantation.

PubMed

Fakhfakh, Raouia; Lamarre, Yann; Skuk, Daniel; Tremblay, Jacques P

2012-01-01

Duchenne muscular dystrophy is a recessive X-linked genetic disease caused by dystrophin gene mutations. Cell therapy can be a potential approach aiming to introduce a functional dystrophin in the dystrophic patient myofibers. However, this strategy produced so far limited results. Transforming growth factor-β (TGF-β) is a negative regulator of skeletal muscle development and is responsible for limiting myogenic regeneration. The combination of TGF-β signaling inhibition with myoblast transplantation can be an effective therapeutic approach in dystrophin-deficient patients. Our aim was to verify whether the success of human myoblast transplantation in immunodeficient dystrophic mice is enhanced with losartan, a molecule that downregulates TGF-β expression. In vitro, blocking TGF-β activity with losartan increased proliferation and fusion and decreased apoptosis in human myoblasts. In vivo, human myoblasts were transplanted in mice treated with oral losartan. Immunodetection of human dystrophin in tibialis anterior cross sections 1 month posttransplantation revealed more human dystrophin-positive myofibers in these mice than in nontreated dystrophic mice. Thus, blocking the TGF-β signal with losartan treatment improved the success of myoblast transplantation probably by increasing myoblast proliferation and fusion, decreasing macrophage activation, and changing the expression of myogenic regulator factors.

Radiosurgery-induced anterior inferior cerebellar artery pseudoaneurysm treated with trapping and bypass.

PubMed

Umekawa, Motoyuki; Hasegawa, Hirotaka; Shin, Masahiro; Kawashima, Mariko; Nomura, Seiji; Nakatomi, Hirofumi; Saito, Nobuhito

2018-05-02

Stereotactic radiosurgery (SRS) is an established modality for the treatment of vestibular schwannomas (VSs). However, its long-term vascular complications have not been well-studied. Among the 360 patients who underwent SRS for VS in our institution and lived for >5 years thereafter, we identified only 1 patient who exhibited complication due to a late-onset aneurysm; thus, the incidence was roughly estimated to be 0.3%. The patient was a 78-year-old man who had undergone SRS for a right VS, and presented with right peripheral facial palsy 19 years later. Radiographic examinations revealed a distal anterior inferior cerebellar artery (AICA) fusiform aneurysm, which was embedded in the tumor, and progressively enlarged over 17 months. Although the right AICA perfused a large area of the cerebellum, the aneurysm was successfully treated with AICA trapping, in conjunction with an occipital artery-AICA bypass. Distal AICA pseudoaneurysm formation is a rare, but potentially severe, late complication after SRS for VS. This is the first case that successfully treated this pathology with AICA trapping with occipital artery-AICA bypass. Copyright © 2018. Published by Elsevier Inc.

Medium voltage therapy for preventing and treating asystole and PEA in ICDs.

PubMed

Gilman, Byron L; Brewer, James E; Kroll, Kai; Kroll, Mark W

2009-01-01

Sudden cardiac death (SCD) takes up to 500,000 lives each year before a victim can even be treated. To address this the implantable cardioverter defibrillator (ICD) was developed to treat those identified at high risk of SCD. Unfortunately, there are a significant number of cases in which the ICD does not successfully return a victim to normal rhythm and effective perfusion of the blood. The vast majority of cases that are not responsive to the ICD therapy require cardio-pulmonary resuscitation (CPR) according to current resuscitation guidelines. A novel electrical stimulus called medium voltage therapy (MVT) has shown efficacy in producing coronary and carotid blood flow during ventricular fibrillation. This report presents the case that the same stimulus may be effective and feasible for use in ICD patients that do not respond to their ICD therapy, or do not have a rhythm in which, an ICD shock is indicated. The inclusion of MVT technology in implantable devices may be effective in preparing the heart for successful defibrillation or in improving the metabolic condition of the heart to the extent that a pulsatile rhythm may spontaneously develop.

Treatment of Recurrent Dupuytren Contracture in Joints Previously Effectively Treated With Collagenase Clostridium histolyticum.

PubMed

Bear, Brian J; Peimer, Clayton A; Kaplan, F Thomas D; Kaufman, Gregory J; Tursi, James P; Smith, Ted

2017-05-01

Collagenase Clostridium histolyticum (CCH) is approved for the treatment of adults with Dupuytren contracture with a palpable cord. This open-label, phase 4 study evaluated the safety and efficacy of CCH for the retreatment of recurrent contractures in joints that were previously effectively treated with CCH. Patients participating in a long-term follow-up study who had contracture recurrence (increased ≥ 20° with a palpable cord) after successful treatment in the previous study were eligible. Recurrent joint contractures were treated with up to 3 CCH injections (∼ 1 month apart). Patients were followed for 1 year to evaluate safety. Assessments included change in joint contracture, range of motion, and the percentage of joints that achieved contracture of 5° or less at day 30 after the last injection. The efficacy analysis included 51 patients with 1 treated joint per patient (31 metacarpophalangeal, 20 proximal interphalangeal). A total of 35 joints (69%) received 1 injection, 12 (24%) received 2 injections, and 4 (8%) received 3 injections. Fifty-seven percent of joints achieved contracture of 5° or less (29 of 51). Overall, 86% (43 of 50) patients had a 20° or greater increase in range of motion. The adverse event profile was consistent with previous studies. One ligament injury was reported. At a short-term follow-up of 1 year, recurrent contracture in joints previously successfully treated with CCH may be effectively retreated with up to 3 injections of CCH. Therapeutic IV. Copyright © 2017 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.

Authoritative parenting, psychosocial maturity, and academic success among adolescents.

PubMed

Steinberg, L; Elmen, J D; Mounts, N S

1989-12-01

The over-time relation between 3 aspects of authoritative parenting--acceptance, psychological autonomy, and behavioral control--and school achievement was examined in a sample of 120 10-16-year-olds in order to test the hypothesis that authoritative parenting facilitates, rather than simply accompanies, school success. In addition, the mediating role of youngsters' psychosocial maturity was studied. Results indicate that (1) authoritative parenting facilitates adolescents' academic success, (2) each component of authoritativeness studied makes an independent contribution to achievement, and (3) the positive impact of authoritative parenting on achievement is mediated at least in part through the effects of authoritativeness on the development of a healthy sense of autonomy and, more specifically, a healthy psychological orientation toward work. Adolescents who describe their parents as treating them warmly, democratically, and firmly are more likely than their peers to develop positive attitudes toward, and beliefs about, their achievement, and as a consequence, they are more likely to do better in school.

A new technique to treat facet joint pain with pulsed radiofrequency.

PubMed

Schianchi, Pietro Martino

2015-02-01

Facet joint pain affects 5% to 15% of the population with low back pain and the prevalence increases with age due to progression of arthritis. While conservative treatments are often unsuccessful, the scientific evidence on minimally invasive therapies such as intra-articular steroid infiltration and continuous and pulsed radiofrequency (PRF) of the medial branches is contradictory. Since PRF has recently been reported to successfully treat joint pain, a new application of this method is proposed for facetogenic lumbar pain via an intra-articular subcapsular approach. Here we reported two cases with successful treatment. A 71-year-old patient presented because of persisting pain in the left gluteal region radiating to the lateral thigh and calf when standing. Anti-inflammatory drugs produced only short-lasting insufficient relief. A 52-year-old employee was admitted in June 2012 because of axial lower lumbar pain with intermittent diffuse radiation to the right lower extremity that worsened during walking and lying down despite receiving analgesics and physiotherapy. A new approach to treat lumbar facet joint pain with PRF is simple to perform and without serious complications. In view of the good long-lasting results obtained with the two reported cases, randomized control trials are necessary to validate this new approach.

Successful treatment of heart failure in an adult patient with Prader-Willi syndrome.

PubMed

Kawano, Hiroaki; Ikeda, Tooru; Shimazaki, Koichi; Arakawa, Shuji; Matsumoto, Yuji; Hayano, Motonobu; Maemura, Koji

2013-01-01

Prader-Willi Syndrome (PWS) is a rare genetic disorder characterized by physical, psychological and physiological abnormalities. Obesity and related cardiovascular diseases are a common problem in adult patients with PWS. This report describes a case of adult PWS with heart failure associated with marked obesity and sleep-disordered breathing that was successfully treated with oxygen therapy, adaptive servoventilation, medications, diet therapy and rehabilitation.

Towards the differentiation of non-treated and treated corundum minerals by ion-beam-induced luminescence and other complementary techniques.

PubMed

Calvo del Castillo, H; Deprez, N; Dupuis, T; Mathis, F; Deneckere, A; Vandenabeele, P; Calderón, T; Strivay, D

2009-06-01

(part of them treated as explained above). The PIXE elemental concentrations of the samples showed large quantities of calcium and lead in some cases that can be linked to treatment with fluxes or lead oxide. The plot of the chromium and iron concentration grouped the samples in various aggregates that corresponded to the different types of corundum analysed. Micro-Raman complemented the PIXE analysis corroborating the presence of lead oxides but the use of the PIGE technique was not successful for the detection of beryllium due to the low cross section of the nuclear reaction chosen for its identification. IBIL was capable of distinguishing between treated and non-treated samples of the same type based on the luminescent features of the materials.

Oxytocin and Naltrexone Successfully Treat Hypothalamic Obesity in a Boy Post-Craniopharyngioma Resection.

PubMed

Hsu, Eugenie A; Miller, Jennifer L; Perez, Francisco A; Roth, Christian L

2018-02-01

Hypothalamic obesity, a treatment-resistant condition common to survivors of craniopharyngioma (CP), is strongly associated with a poor quality of life in this population. Oxytocin (OT), a hypothalamic neuropeptide, has been shown to play a role in the regulation of energy balance and to have anorexigenic effects in animal studies. Naltrexone (NAL), an opiate antagonist, has been shown to deter hedonic eating and to potentiate OT's effects. In this parent-observed study, we tested the administration of intranasal OT for 10 weeks (phase 1), followed by a combination of intranasal OT and NAL for 38 weeks (phase 2) in a 13-year-old male with confirmed hypothalamic obesity and hyperphagia post-CP resection. Treatment resulted in 1) reduction in body mass index (BMI) z score from 1.77 to 1.49 over 10 weeks during phase 1; 2) reduction in BMI z score from 1.49 to 0.82 over 38 weeks during phase 2; 3) reduced hyperphagia during phases 1 and 2; 4) continued hedonic high-carbohydrate food-seeking in the absence of hunger during phases 1 and 2; and 5) sustained weight reduction during decreased parental monitoring and free access to unlocked food in the home during the last 10 weeks of phase 2. This successful intervention of CP-related hypothalamic obesity and hyperphagia by OT alone and in combination with NAL is promising for conducting future studies of this treatment-recalcitrant form of obesity. Copyright © 2017 Endocrine Society

Treat the whole patient and be aware of drug interactions.

PubMed

Breivik, Harald

2015-03-01

The case of an elderly male with bilateral shoulder pain is presented. The pain had been successfully treated years earlier with surgery, but a repeat rotator cuff procedure when the pain recurred was not effective. The patient's physician asked about impact of systemic analgesics on the elderly patient and interactions with his blood pressure medications. Cardiovascular and renal risks of NSAOIDs are discussed as are potential toxicities of tramadol and too rapid withdrawal from it. Drug interactions of medications used are described.

Clinical Development of Cell Therapies: Setting the Stage for Academic Success.

PubMed

Abou-El-Enein, M; Volk, H-D; Reinke, P

2017-01-01

Cellular therapies have potential to treat a wide range of diseases with autologous immunotherapies showing unprecedented therapeutic promise in clinical trials. Such therapies are mainly developed by academic researchers applying small-scale production, targeting rare and unmet medical needs. Here, we highlight the clinical translation of immunotherapy product in an academic setting, which may serve as a success model for early academic development of cell-based therapeutics. © 2016 American Society for Clinical Pharmacology and Therapeutics.

Anti-Müllerian hormone as a predictor of polycystic ovary syndrome treated with clomiphene citrate

PubMed Central

Negoro, Yuwono Sri; Afrita, Yohana; Wiweko, Budi; Sumapradja, Kanadi; Natadisastra, Muharam

2016-01-01

Objective This study aimed to determine the threshold of anti-Müllerian hormone (AMH) as predictor of follicular growth failure in polycystic ovary syndrome (PCOS) patients treated with clomiphene citrate (CC). Methods Fifty female subjects with PCOS were recruited and divided into two groups based on successful and unsuccessful follicular growth. Related variables such as age, infertility duration, cigarette smoking, use of Moslem hijab, sunlight exposure, fiber intake, body mass index, waist circumference, AMH level, 25-hydroxy vitamin D level, and growth of dominant follicles were obtained, assessed, and statistically analyzed. Results The AMH levels of patients with successful follicular growth were significantly lower (p=0.001) than those with unsuccessful follicular growth (6.10±3.52 vs. 10.43±4.78 ng/mL). A higher volume of fiber intake was also observed in the successful follicular growth group compared to unsuccessful follicular growth group (p=0.001). Our study found the probability of successful follicle growth was a function of AMH level and the amount of fiber intake, expressed as Y=–2.35+(–0.312×AMH level)+(0.464×fiber intake) (area under the curve, 0.88; 95% confidence interval, 0.79–0.98; p<0.001). Conclusion The optimal threshold of AMH level in predicting the failure of follicle growth in patients with PCOS treated with CC was 8.58 ng/mL. PMID:28090459

EUS-guided drainage is more successful in pancreatic pseudocysts compared with abscesses

PubMed Central

Sadik, Riadh; Kalaitzakis, Evangelos; Thune, Anders; Hansen, Jan; Jönson, Claes

2011-01-01

AIM: To compare the results for endoscopic ultrasound (EUS)-guided drainage of clear fluid pancreatic pseudocysts with the results for abscess drainage. METHODS: All patients referred for endoscopic drainage of a fluid collection were prospectively included. The outcome was recorded. RESULTS: Altogether 26 pseudocysts or abscesses were treated in 25 (6 female) patients. One endoscopist performed the procedures. Non-infected pseudocysts were present in 15 patients and 10 patients had infected fluid collections. The cyst size ranged between 28 cm × 13 cm and 5 cm × 5 cm. The EUS drainage was successful in 94% of the pseudocysts and in 80% of the abscesses (P = 0.04). The complication rate in pseudocysts was 6% and in abscesses was 30% (P = 0.02). Recurrence of a pseudocyst occurred in one patient (4%) after 6 mo; the patient was successfully retreated. CONCLUSION: EUS-guided drainage of pseudocysts is associated with a higher success rate and a lower complication rate compared with abscess drainage. PMID:21274380

Basiliximab #2: In-Vivo Activated T-Cell Depletion to Prevent Graft-Versus_Host Disease (GVHD) After Nonmyeloablative Allotransplantation for the Treatment of Blood Cancer

ClinicalTrials.gov

2016-01-28

Acute Myelogenous Leukemia; Acute Lymphocytic Leukemia; Chronic Myelogenous Leukemia; Chronic Lymphocytic Leukemia; Myelodysplasia; Non-Hodgkin's Lymphoma; Hodgkin's Disease; Multiple Myeloma; Myelofibrosis; Anemia, Aplastic; Hemoglobinuria, Paroxysmal

Phase I Dose-Escalation Trial of Clofarabine Followed by Escalating Doses of Fractionated Cyclophosphamide in Children With Relapsed or Refractory Acute Leukemias

ClinicalTrials.gov

2018-02-13

Myelodysplastic Syndrome; Acute Myeloid Leukemia; Myeloproliferative Disorders; Acute Lymphocytic Leukemia; Acute Promyelocytic Leukemia; Acute Leukemia; Chronic Myelogenous Leukemia; Myelofibrosis; Chronic Myelomonocytic Leukemia; Juvenile Myelomonocytic Leukemia

Cost per successfully treated patient for vortioxetine versus duloxetine in adults with major depressive disorder: an analysis of the complete symptoms of depression and functional outcome.

PubMed

Christensen, Michael Cronquist; Munro, Vicki

2018-04-01

To determine the cost-effectiveness of vortioxetine vs duloxetine in adults with moderate-to-severe major depressive disorder (MDD) in Norway using a definition of a successfully treated patient (STP) that incorporates improvement in both mood symptoms and functional capacity. Using the population of patients who completed the 8-week CONNECT study, the cost-effectiveness of vortioxetine (n = 168) (10-20 mg/day) vs duloxetine (n = 176) (60 mg/day) was investigated for the treatment of adults in Norway with moderate-to-severe MDD and self-reported cognitive dysfunction over an 8-week treatment period. Cost-effectiveness was assessed in terms of cost per STP, defined as improvement in mood symptoms (≥50% decrease from baseline in Montgomery-Åsberg Depression Rating Scale total score) and change in UCSD [University of California San Diego] performance-based skills assessment [UPSA] score of ≥7. The base case analysis utilized pharmacy retail price (apotek utsalgspris (AUP)) for branded vortioxetine (Brintellix) and branded duloxetine (Cymbalta). After 8 weeks of antidepressant therapy, there were more STPs with vortioxetine than with duloxetine (27.4% vs 22.5%, respectively). The mean number needed to treat for each STP was 3.6 for vortioxetine and 4.4 for duloxetine, resulting in a lower mean cost per STP for vortioxetine (NOK [Norwegian Kroner] 3264) than for duloxetine (NOK 3310) and an incremental cost per STP of NOK 3051. The use of a more challenging change in the UPSA score from baseline (≥9) resulted in a mean cost per STP of NOK 3822 for vortioxetine compared with NOK 3983 for duloxetine and an incremental cost per STP of NOK 3181. Vortioxetine may be a cost-effective alternative to duloxetine, owing to its superior ability to improve functional capacity. The dual-response STP concept introduced here represents a more comprehensive analysis of the cost-effectiveness of antidepressants.

Yes I can: Expected success promotes actual success in emotion regulation.

PubMed

Bigman, Yochanan E; Mauss, Iris B; Gross, James J; Tamir, Maya

2016-11-01

People who expect to be successful in regulating their emotions tend to experience less frequent negative emotions and are less likely to suffer from depression. It is not clear, however, whether beliefs about the likelihood of success in emotion regulation can shape actual emotion regulation success. To test this possibility, we manipulated participants' beliefs about the likelihood of success in emotion regulation and assessed their subsequent ability to regulate their emotions during a negative emotion induction. We found that participants who were led to expect emotion regulation to be more successful were subsequently more successful in regulating their emotional responses, compared to participants in the control condition. Our findings demonstrate that expected success can contribute to actual success in emotion regulation.

oVEMP as an objective indicator of successful repositioning maneuver.

PubMed

Asal, Samir; Sobhy, Osama; Balbaa, Amany

Benign paroxysmal positioning vertigo (BPPV) is the most common peripheral vestibular disorder. Canalolithiasis in the posterior semi-circular canal is the most common underlying pathology that can be treated effectively by repositioning maneuvers. Our hypothesis suggested that successful maneuvers can lead to repositioning of dislodged otoconia to the utricle. Air conducted oVEMP, which is thought to originate from the contra-lateral utricular organ was measured in twenty patients with unilateral BPPV and we compared n1-p1 peak to peak amplitude of the affected ears in 3 separate intervals: on pre-treatment when typical nystagmus was confirmed, immediately after, and 1 week after repositioning maneuvers to assess change, if any, in amplitude. This study showed significant increase of oVEMP amplitude in the affected ears after successful repositioning maneuver that was more significant after 1 week. oVEMP can be used as a reliable objective test for ensuring a successful maneuver rather than subjective dependence on the patient's symptoms, which may be misleading due to a remission. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

Efficacy of spinal cord stimulators in treating peripheral neuropathy: a case series.

PubMed

Abd-Elsayed, Alaa; Schiavoni, Nick; Sachdeva, Harsh

2016-02-01

Peripheral neuropathy is a common cause of pain, and it is increasing in prevalence. Peripheral neuropathic pain is very hard to treat and can be resistant to multiple pain management modalities. Our series aimed at testing the efficacy of spinal cord stimulators (SCSs) in treating resistant painful peripheral neuropathy. Case 1: A 79-year-old man presented to our clinic with long-standing history of painful peripheral diabetic neuropathy resistant to conservative management. After failure of all possible modalities, we offered the patient an SCS trial that was very successful, and we proceeded with the permanent implant that continued to help with his pain and allowed the patient to wean down his medications. Case 2: A 60-year-old man presented with chronic peripheral neuropathy secondary to HIV, patient failed all conservative and procedural management. Patient then had an SCS trial that relieved his pain significantly. Unfortunately, we did not proceed with the implant due to deterioration of the patient general health. Case 3: A 39-year-old woman presented with painful peripheral neuropathy secondary to chemotherapy for breast cancer. After failure of medication management and procedures, patient had a SCS trial that improved her pain and we then proceeded with performing the permanent implant that controlled her pain. We presented 3 cases with chronic painful peripheral neuropathy secondary to HIV, diabetes mellitus, and chemotherapy that was resistant to conservative pain management and procedures that was successfully treated with neurostimulation. Copyright © 2016 Elsevier Inc. All rights reserved.

Impact of upper airway abnormalities on the success and adherence to mandibular advancement device treatment in patients with Obstructive Sleep Apnea Syndrome.

PubMed

Prescinotto, Renato; Haddad, Fernanda Louise Martinho; Fukuchi, Ilana; Gregório, Luiz Carlos; Cunali, Paulo Afonso; Tufik, Sérgio; Bittencourt, Lia Rita Azeredo

2015-01-01

The mandibular advancement device (MAD) is a option to treat patients with Obstructive Sleep Apnea Syndrome (OSAS). To assess the influence of upper airway abnormalities on the success of and adherence to MAD in patients with OSAS. Prospective study with 30 patients with mild to moderate OSAS and indications for MAD. The protocol included questionnaires addressing sleep and nasal complaints, polysomnography, and upper airway assessment. The analyzed parameters of patients who showed therapeutic success and failure and those who exhibited good and poor treatment adherence were compared. 28 patients completed the protocol; 64.3% responded successfully to treatment with MAD, and 60.7% exhibited good adherence to treatment. Factors associated with greater success rates were younger age (p=0.02), smaller cervical circumference (p=0.05), and lower AHI at baseline (p=0.05). There was a predominance of patients without nasal abnormalities among patients treated successfully compared to those with treatment failure (p=0.04), which was not observed in relation to adherence. Neither pharyngeal nor facial skeletal abnormalities were significantly associated with either therapeutic success or adherence. MAD treatment success was significantly lower among patients with nasal abnormalities; however, treatment adherence was not influenced by the presence of upper airway or facial skeletal abnormalities. Copyright © 2015 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

A comprehensive protocol to diagnose and treat pain of muscular origin may successfully and reliably decrease or eliminate pain in a chronic pain population.

PubMed

Marcus, Norman J; Gracely, Edward J; Keefe, Kelly O

2010-01-01

A comprehensive protocol is presented to identify muscular causes of regional pain syndromes utilizing an electrical stimulus in lieu of palpation, and combining elements of Prolotherapy with trigger point injections. One hundred seventy-six consecutive patients were evaluated for the presence of muscle pain by utilizing an electrical stimulus produced by the Muscle Pain Detection Device. The diagnosis of "Muscle Pain Amenable to Injection" (MPAI), rather than trigger points, was made if pain was produced for the duration of the stimulation. If MPAI was found, muscle tendon injections (MTI) were offered to patients along with post-MTI physical therapy, providing neuromuscular electrical stimulation followed by a validated exercise program [1]. A control group, evaluated 1 month prior to their actual consultation/evaluation when muscle pain was identified but not yet treated, was used for comparison. Forty-five patients who met criteria completed treatment. Patients' scores on the Brief Pain Inventory decreased an average of 62%; median 70% (P < 0.001) for pain severity and 68%; median 85% (P < 0.001) for pain interference one month following treatment. These changes were significantly greater (P < 0.001) than those observed in the untreated controls. A protocol incorporating an easily reproducible electrical stimulus to diagnose a muscle causing pain in a region of the body followed by an injection technique that involves the entirety of the muscle, and post injection restoration of muscle function, can successfully eliminate or significantly reduce regional pain present for years.

Successful Use of Orbital Atherectomy as an Adjunct in Treating Extensively Calcified Mesenteric Artery Lesions.

PubMed

Richard, Michele; Krol, Emilia; Dietzek, Alan

2016-10-01

To describe the use of orbital technique of atherectomy as an adjunct to successful angioplasty and stent placement of the superior mesenteric artery (SMA). The technique is demonstrated in a 68-year-old man with critical SMA stenosis. The SMA was cannulated with 0.014-in wire, but the lesion was highly stenotic and densely calcified and prevented the passage of even the smallest 1.5-mm balloon. Orbital atherectomy was thus performed with a 1.25-mm CSI crown. Balloon angioplasty was then possible with a 4 mm × 2 cm balloon followed by placement of a 7 mm × 22 mm balloon-mounted stent. The use of atherectomy as an adjunct to angioplasty and stenting in extensive, calcified SMA lesions supports the value of this technique to avoid a much larger and morbid open procedure. Published by Elsevier Inc.

Ocular toxicity in BRAF mutant cutaneous melanoma patients treated with vemurafenib.

PubMed

Choe, Christina H; McArthur, Grant A; Caro, Ivor; Kempen, John H; Amaravadi, Ravi K

2014-10-01

To determine the frequency of ocular adverse effects associated with vemurafenib (PLX4032) treatment for metastatic cutaneous melanoma. Retrospective review of the clinical study reports from the clinical pharmacology, phase 1, phase 2, and phase 3 trials of vemurafenib. The vemurafenib clinical trials were a multicenter series involving adult patients with histologically confirmed, BRAF(V600) mutation-positive, unresectable, stage IIIC or IV melanoma. A total of 855 patients were enrolled in the trials: 568 patients were treated with vemurafenib and 287 patients were treated with dacarbazine. Among the 568 patients treated with vemurafenib, ocular adverse effects developed in 22% (95% confidence interval [CI], 18.5-25.6). The most common ocular diagnosis was uveitis (4.0%; 95% CI, 2.6-6.0), followed by conjunctivitis (2.8%; 95% CI, 1.6-4.5) and dry eyes (2.0%; 95% CI, 1.1-3.7). All were successfully managed while vemurafenib therapy was continued. Ocular adverse events and symptoms may be seen in more than one-fifth of patients being treated with vemurafenib. However, vemurafenib can be continued while the ocular symptoms are being managed. The pathogenesis of ocular symptoms in this patient population is unclear; additional studies are necessary. Copyright © 2014 Elsevier Inc. All rights reserved.

Bridge Suture for Successful McDonald Emergency Cerclage.

PubMed

Tanaka, Masaaki; Hori, Yoshiaki; Shirafuji, Aya; Kato, Mitsunori; Kato, Jyun; Kobayashi, Hiroto; Tsuchida, Toru; Fukae, Tsukasa

2017-01-01

To create awareness about a surgical technique termed bridge suture, which is performed as a pretreatment before a McDonald cerclage is performed on an emergency to treat severe cervical insufficiency. Procedures for bridge suture were reviewed in detail and outcomes of 16 patients treated with bridge suture followed by McDonald cerclage were evaluated retrospectively. Using the bridge suture, the edges of uterine cervix were temporarily sutured and the external uterine os was closed, while the hourglass-shaped fetal membranes were concomitantly confined within the cervix; subsequently, a McDonald cerclage was performed. Over a 22-year period, 16 patients with a dilated cervix and bulging fetal membranes were treated using the technique of bridge suture followed by an emergency cerclage. The mean gestational age at cerclage was 22.5 weeks; the mean gestational age at delivery was 30.7 weeks; and the mean interval between cerclage and delivery was 8.2 weeks. In 15 out of 16 cases, cerclage was performed without encountering any complications. No maternal complications, including cervical laceration, were observed. The mean body weight of 17 neonates, including that of a twin, was 1,516 g and of them, 15 neonates survived. The important outcome of bridge suture is the replacement of fetal membranes back into the uterine cavity before McDonald's cerclage is performed. Pretreatment with bridge suture may facilitate the performance of a successful emergency cerclage and contribute to good maternal and neonatal outcomes. © 2016 S. Karger AG, Basel.

Intrahepatic Flow Redistribution in Patients Treated with Radioembolization

DOE Office of Scientific and Technical Information (OSTI.GOV)

Spreafico, Carlo, E-mail: carlo.spreafico@istitutotumori.mi.it; Morosi, Carlo, E-mail: carlo.morosi@istitutotumori.mi.it; Maccauro, Marco, E-mail: marco.maccauro@istitutotumori.mi.it

2015-04-15

IntroductionIn planning Yttrium-90 ({sup 90}Y)-radioembolizations, strategy problems arise in tumours with multiple arterial supplies. We aim to demonstrate that tumours can be treated via one main feeding artery achieving flow redistribution by embolizing accessory vessels.MethodsOne hundred {sup 90}Y-radioembolizations were performed on 90 patients using glass microspheres. In 19 lesions/17 patients, accessory branches were found feeding a minor tumour portion and embolized. In all 17 patients, the assessment of the complete perfusion was obtained by angiography and single photon emission computerized tomography–computerized tomography (SPECT–CT). Dosimetry, toxicity, and tumor response rate of the patients treated after flow redistribution were compared with themore » 83 standard-treated patients. Seventeen lesions in 15 patients with flow redistribution were chosen as target lesions and evaluated according to mRECIST criteria.ResultsIn all patients, the complete tumor perfusion was assessed immediately before radioembolization by angiography in all patients and after the {sup 90}Y-infusion by SPECT–CT in 15 of 17 patients. In the 15 assessable patients, the response rate in their 17 lesions was 3 CR, 8 PR, and 6 SD. Dosimetric and toxicity data, as well tumour response rate, were comparable with the 83 patients with regular vasculature.ConclusionsAll embolization procedures were performed successfully with no complications, and the flow redistribution was obtained in all cases. Results in term of toxicity, median dose administered, and radiological response were comparable with standard radioembolizations. Our findings confirmed the intratumoral flow redistribution after embolizing the accessory arteries, which makes it possible to treat the tumour through its single main feeding artery.« less

Behaviour of Anopheles albimanus in relation to pyrethroid-treated bednets.

PubMed

Arredondo-Jiménez, J I; Rodríguez, M H; Loyola, E G; Bown, D N

1997-01-01

Responses of the malaria vector Anopheles albimanus to pyrethroid impregnated bednets made of cotton or nylon, compared with untreated nets, were investigated in houses occupied by two people inside and/or outside two bednets, in coastal Chiapas, México. The pyrethroid used was lambdacyhalothrin 30 mg a.i./m2. Bioassay mortality rates of An.albimanus exposed to treated nets for 3 or 15 min, rose from 40-55% to 90-100% for nylon nets 3-19 weeks post-treatment, but were consistently lower for treated cotton nets. An.albimanus females (collected unfed on human bait) were released in houses surrounded by curtains for trapping mosquitoes that exited from the house. Floor sheets were used in and around each experimental house for retrieving any mosquitoes knocked-down and/or killed. During post-treatment assessment for 17 weeks, An.albimanus blood-feeding success rates were 23-24% with untreated nets, 14-18% with treated cotton nets and 8-15% with treated nylon nets, significantly reduced when both human baits were inside the treated bednets, but not when one or more baits were outside the treated bednet(s) within the house. Proportions of mosquitoes leaving houses < 3 h post-release were 53-59% from houses with untreated bednets versus 65-78% with treated bednets. Except in one case (when both humans were outside treated cotton nets), these increased early exit rates were significant, whether or not the human baits were inside the treated bednets indoors. Mortality rates of An.albimanus females exiting overnight (22.00-06.00 hours) averaged 15-39% from houses with treated cotton and 16-46% with treated nylon nets, very significantly greater than the control mean rates of 6-8% mortality with untreated nets. Observations on wild-caught An.albimanus females marked with fluorescent powder and released indoors revealed that few mosquitoes (3-11%) actually contacted the bednets unless both human baits remained under them - when contact rates were 22% on treated nylon, 23% on

Punica granatum: A review on its potential role in treating periodontal disease

PubMed Central

Prasad, Divyashree; Kunnaiah, Ravi

2014-01-01

A wide variety of plants possess enormous treasure of medicinal value. Majority of these medicinal plants have been used to treat various systemic conditions successfully. Over the recent years, use of these medicinal plants has resurfaced to treat oral conditions. Among the oral conditions, periodontal disease remains one of the most common. Alternative and preventive options has become the need of the hour in order to overcome the adverse effects of the antimicrobial agents used in large as an adjunct to mainstream periodontal treatment. Punica granatum (Pomegranate) is one of the oldest edible fruit which has a long history as a medicinal fruit. This review is an attempt to highlight the potential of Punica granatum as a preventive and therapeutic aid to periodontal disease. PMID:25210254

Successful use of heat as first aid for tropical Australian jellyfish stings.

PubMed

Little, Mark; Fitzpatrick, Richard; Seymour, Jamie

2016-11-01

Currently the Australian Resuscitation Council (ARC) recommends dousing with vinegar followed by ice as first aid for jellyfish stings in tropical Australia, with limited evidence to support this recommendation (Li et al., 2013). We report our successful experience in using hot water immersion as first aid in treating two people stung by venomous tropical Australian jellyfish, one by Chironex fleckeri and one by Carukia barnesi. Copyright © 2016 Elsevier Ltd. All rights reserved.

Perceived neighborhood safety, recovery capital, and successful outcomes among mothers 10 years after substance abuse treatment.

PubMed

Evans, Elizabeth; Li, Libo; Buoncristiani, Samantha; Hser, Yih-Ing

2014-09-01

This study examines perceived neighborhood characteristics associated with successful outcome among mothers 10 years after being treated for substance use disorders. Data were obtained from 713 mothers first studied at admission to drug treatment in California in 2000-2002 and followed up in 2009-2011. At follow-up, 53.6% of mothers had a successful outcome (i.e., no use of illicit drugs and not involved with the criminal justice system). Perceived neighborhood safety almost doubled the odds of success. Perceived neighborhood safety interacted with social involvement, decreasing the odds of success among mothers who reported more versus less neighborhood social involvement. Perceived neighborhood climate is associated with long-term outcomes among mothers with substance use disorders independent of individual-level characteristics, underscoring the need for further efforts to understand its interaction with recovery capital in ways that promote and impede health.

Clinical and radiographic success of mineral trioxide aggregate compared with formocresol as a pulpotomy treatment in primary molars: a systematic review and meta-analysis.

PubMed

Marghalani, Abdullah A; Omar, Samah; Chen, Jung-Wei

2014-07-01

The authors conducted a systematic review and meta-analysis to compare the long-term clinical and radiographic success of using mineral trioxide aggregate (MTA) and formocresol (FC) as a pulp-dressing material in pulpotomy treatment in primary molars. The authors searched MEDLINE, Thomson Reuters Web of Science and the Cochrane Central Register of Controlled Trials for randomized controlled trials (RCTs) published from Jan. 1, 1990, to May 9, 2013. For an RCT to be included, the authors required that the primary molars treated with a pulpotomy procedure must have received stainless steel crowns as a final restoration and that rubber dam isolation was used during treatment; that the pulp must have been vital as determined clinically by means of hemorrhage control with a cotton pellet; and that the RCT must have included a follow-up period of at least two years. For each included RCT, two authors assessed the risk of bias independently. The authors identified 20 trials and included five of them. A total of 377 primary molars were treated. The authors judged that none of the included RCTs had a low risk of bias. They noted no significant differences in clinical success (relative risk [RR] = 1.01; 95 percent confidence interval [CI], 0.98-1.05) and radiographic success (RR = 1.09; 95 percent CI, 0.97-1.21) for primary molars treated with MTA versus those treated with FC. On the basis of the limited evidence, pulpotomy procedures performed in primary molars involving the use of MTA or FC showed comparable clinical success rates.

Osteopathic lymphatic pump techniques to enhance immunity and treat pneumonia.

PubMed

Hodge, Lisa M

2012-03-01

Pneumonia is a common cause of morbidity and mortality worldwide. While antibiotics are generally effective for the treatment of infection, the emergence of resistant strains of bacteria threatens their success. The osteopathic medical profession has designed a set of manipulative techniques called lymphatic pump techniques (LPT), to enhance the flow of lymph through the lymphatic system. Clinically, LPT is used to treat infection and oedemaand might be an effective adjuvant therapy in patients with pneumonia.The immune system uses the lymphatic and blood systems to survey to rid the body of pathogens; however, only recently have the effects of LPT on the lymphatic and immune systems been investigated. This short review highlightsclinical and basic science research studies that support the use of LPT to enhance the lymphatic and immune systems and treat pneumonia, and discusses the potential mechanisms by which LPT benefits patients with pneumonia.

Bone marrow aspiration

MedlinePlus

Iliac crest tap; Sternal tap; Leukemia - bone marrow aspiration; Aplastic anemia - bone marrow aspiration; Myelodysplastic syndrome - bone marrow aspiration; Thrombocytopenia - bone marrow aspiration; Myelofibrosis - bone marrow aspiration

Experience with ruxolitinib in the treatment of polycythaemia vera

PubMed Central

Alimam, Samah; Harrison, Claire

2017-01-01

Polycythaemia vera (PV) is a myeloproliferative neoplasm classically characterized by an erythrocytosis and is associated with a high risk of thromboembolic events, constitutional symptoms burden and risk of transformation to myelofibrosis and acute myeloid leukaemia. Therapy is directed at the haematocrit (HCT) to reduce the risk of thrombotic events and usually comprises low-dose aspirin and phlebotomy to maintain HCT at >45%. Frequently in addition, cytoreductive therapy is indicated in high-risk patients for normalizing haematological parameters to mitigate the occurrence of thromboembolic events. Unfortunately, there is no clear evidence that current therapies reduce the risk of transformation to myelofibrosis and for some a risk of a therapy related complication is unknown for example leukaemia due to hydroxycarbamide (HC). First-line therapy for treating PV remains HC or interferon, the latter most often in younger patients, especially those of childbearing age. However, therapy related intolerance or resistance is a common feature and results in limited treatment options for such patients. The discovery of the JAK2 V617F mutation and consequently targeted therapy with Janus kinase inhibitors, in particular ruxolitinib, has extended the spectrum of agents that can be used as second or third line in PV. The findings of the phase II trial RESPONSE and the preliminary data from RESPONSE 2 trial have identified a role for ruxolitinib in PV patients who are resistant or intolerant to HC. In this article, using clinical cases we demonstrate our experience with ruxolitinib highlighting the clinical benefits and limitations we encountered in clinical practice. PMID:28491265

Effect of rebamipide ophthalmic suspension on the success of lacrimal stent intubation.

PubMed

Mimura, Masashi; Ueki, Mari; Oku, Hidehiro; Sato, Bunpei; Ikeda, Tsunehiko

2016-02-01

To evaluate the effect of the postoperative administration of rebamipide ophthalmic suspension on the success rate of lacrimal stent intubation (LSI) for the treatment of primary acquired nasolacrimal duct obstruction (PANDO). This comparative interventional cohort study investigated 110 consecutive patients with PANDO who were treated with LSI and followed up for 12 months postoperatively at one institution. LSI was performed by one surgeon, and all patients received identical postoperative care. Among the total 110 patients, 71 underwent LSI with postoperative administration of rebamipide ophthalmic suspension, and 39 underwent LSI without administration of the suspension. Data related to patient age, gender, laterality, and postoperative administration of rebamipide ophthalmic suspension were collected and used as independent variables, and logistic regression analyses were performed to compare the anatomical success rate at 12 months postoperatively between patients with and without postoperative administration of the suspension. The anatomical success rate of LSI in patients with and without postoperative administration of rebamipide ophthalmic suspension was 90.1 and 69.2 %, respectively. A comparison of these success rates showed statistical significance, in that the rate of treatment success was higher in PANDO patients who underwent LSI with postoperative administration of the suspension [odds ratio (OR), 3.37; P < 0.05]. The findings of this study show that postoperative administration of rebamipide ophthalmic suspension increases the rate of anatomical success in patients who undergo LSI for the treatment of PANDO.

Resolution of bone marrow fibrosis in a patient receiving JAK1/JAK2 inhibitor treatment with ruxolitinib.

PubMed

Wilkins, Bridget S; Radia, Deepti; Woodley, Claire; Farhi, Sarah El; Keohane, Clodagh; Harrison, Claire N

2013-12-01

Ruxolitinib, a JAK1/JAK2 inhibitor, is currently the only pharmacological agent approved for the treatment of myelofibrosis. Approval was based on findings from two phase 3 trials comparing ruxolitinib with placebo (COMFORT-I) and with best available therapy (COMFORT-II) for the treatment of primary or secondary myelofibrosis. In those pivotal trials, ruxolitinib rapidly improved splenomegaly, disease-related symptoms, and quality of life and prolonged survival compared with both placebo and conventional treatments. However, for reasons that are currently unclear, there were only modest histomorphological changes in the bone marrow, and only a subset of patients had significant reductions in JAK2 V617F clonal burden. Here we describe a patient with post-polycythemia vera myelofibrosis who received ruxolitinib at our institution (Guy's and St. Thomas' NHS Foundation Trust, London, United Kingdom) as part of the COMFORT-II study. While on treatment, the patient had dramatic improvements in splenomegaly and symptoms shortly after starting ruxolitinib. With longer treatment, the patient had marked reductions in JAK2 V617F allele burden, and fibrosis of the bone marrow resolved after approximately 3 years of ruxolitinib treatment. To our knowledge, this is the first detailed case report of resolution of fibrosis with a JAK1/JAK2 inhibitor. ClinicalTrials.gov Identifier: NCT00934544.

Molecular analyses of 15,542 patients with suspected BCR-ABL1-negative myeloproliferative disorders allow to develop a stepwise diagnostic workflow

PubMed Central

Schnittger, Susanne; Bacher, Ulrike; Eder, Christiane; Dicker, Frank; Alpermann, Tamara; Grossmann, Vera; Kohlmann, Alexander; Kern, Wolfgang; Haferlach, Claudia; Haferlach, Torsten

2012-01-01

We investigated 15,542 patients with suspected BCR-ABL1- negative myeloproliferative or myelodysplastic/myeloproliferative neoplasm (including 359 chronic myelomonocytic leukemia) by a molecular marker set. JAK2V617F was detected in the suspected categories as follows: polycythemia vera 88.3%, primary myelofibrosis 53.8%, essential thrombocythemia 50.2%, and not further classifiable myeloproliferative neoplasms 38.0%. JAK2 exon 12 mutations were detected in 40.0% JAK2V617F-negative suspected polycythemia vera, MPLW515 mutations in 13.2%JAK2V617F-negative primary myelofibrosis and 7.1% JAK2V617F-negative essential thrombocythemia. TET2 mutations were distributed across all entities but were most frequent in suspected chronic myelomonocytic leukemia (77.8%). CBL mutations were identified in suspected chronic myelomonocytic leukemia (13.9%), primary myelofibrosis (8.0%), and not further classifiable myeloproliferative neoplasm (7.0%). This leads to a stepwise workflow for suspected myeloproliferative neoplasms starting with JAK2V617F and investigating JAK2V617F-negative patients for JAK2 exon 12 or MPL mutations, respectively. In cases in which a myeloproliferative neoplasm cannot be established, analysis for TET2, CBL and EZH2 mutations may be indicated. PMID:22511494

Attitudes of Success.

ERIC Educational Resources Information Center

Pendarvis, Faye

This document investigates the attitudes of successful individuals, citing the achievement of established goals as the criteria for success. After offering various definitions of success, the paper focuses on the importance of self-esteem to success and considers ways by which the self-esteem of students can be improved. Theories of human behavior…

Intractable lung abscess successfully treated with cavernostomy and free omental plombage using microvascular surgery.

PubMed

Shimizu, Junzo; Arano, Yoshihiko; Adachi, Iwao; Ikeda, Chikako; Ishikawa, Norihiko; Ohtake, Hiroshi

2009-11-01

A 68-year-old man, complaining of fever and puriform sputum, was referred to our hospital. A giant abscess was detected in the upper lobe of the right lung. Percutaneous drainage of a lung abscess was carried out. When the pus collected was cultured, Candida was 1+ and Escherichia coli was 2+. Later, it became difficult to control the abscess by drainage, and cavernostomy was selected. The contents of the abscess cavity were removed, and the cavity was opened, followed by exchange of gauze every day. For 14 months after cavernostomy, once-weekly gauze exchange was continued at the outpatient clinic to clean the abscess cavity. Finally, the abscess was filled with a free greater omentum flap, accompanied by microvascular anastomosis. In this way, the intractable lung abscess was successfully cured. Conventionally, surgical treatment, particularly cavernostomy, has been applied only to limited cases when dealing with a lung abscess. Our experience with the present case suggests that surgical treatment, including cavernostomy as one option, should also be considered when dealing with lung abscesses resisting medical treatment and causing compromised respiratory function. To enable maximum utilization of the greater omental flap, which is available in only a limited amount, it seems useful to prepare and graft a free omental flap making use of microvascular surgery.

Success in Science, Success in Collaboration

DOE Office of Scientific and Technical Information (OSTI.GOV)

Johnston, Mariann R.

2016-08-25

This is a series of four different scientific problems which were resolved through collaborations. They are: "Better flow cytometry through novel focusing technology", "Take Off ®: Helping the Agriculture Industry Improve the Viability of Sustainable, Large-Production Crops", "The National Institutes of Health's Models of Infectious Disease Agent Study (MIDAS)", and "Expanding the capabilities of SOLVE/RESOLVE through the PHENIX Consortium." For each one, the problem is listed, the solution, advantages, bottom line, then information about the collaboration including: developing the technology, initial success, and continued success.

Primary mediastinal hemangiopericytoma treated with preoperative embolization and surgery.

PubMed

Kulshreshtha, Pranjal; Kannan, Narayanan; Bhardwaj, Reena; Batra, Swati; Gupta, Srishti

2014-01-01

Hemangiopericytomas are rare tumors originating from vascular pericytes. The mediastinum is an extremely uncommon site with only a few cases reported. Diagnosis is based on histopathology and immunohistochemistry, which differentiates them from synovial sarcoma and solitary fibrous histiocytoma. They have a variable malignant potential. Treatment is mainly surgical extirpation as the role of adjuvant therapy is controversial. Preoperative embolization has been sparingly used. We report a case of primary mediastinal hemangiopericytoma in a 47-year-old man treated successfully with preoperative embolization and surgery. Copyright © 2014 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.

Integrating Hypnosis with Other Therapies for Treating Specific Phobias: A Case Series.

PubMed

Hirsch, Joseph A

2018-04-01

There is a high prevalence of anxiety disorders including specific phobias and panic disorder in the United States and Europe. A variety of therapeutic modalities including pharmacotherapy, cognitive behavioral therapy, systematic desensitization, hypnosis, in vivo exposure, and virtual reality exposure therapy have been applied. No one modality has been entirely successful. There has been only a limited attempt to combine psychological therapies in the treatment of specific phobias and panic disorder and what has been done has been primarily with systematic desensitization or cognitive behavioral therapy along with hypnotherapy. I present two cases of multiple specific phobias that were successfully treated with hypnotherapy combined with virtual reality exposure therapy or in vivo exposure therapy. The rationale for this integrative therapy and the neurobiological constructs are considered.

Test-and-treat approach to HIV/AIDS: a primer for mathematical modeling.

PubMed

Nah, Kyeongah; Nishiura, Hiroshi; Tsuchiya, Naho; Sun, Xiaodan; Asai, Yusuke; Imamura, Akifumi

2017-09-05

The public benefit of test-and-treat has induced a need to justify goodness for the public, and mathematical modeling studies have played a key role in designing and evaluating the test-and-treat strategy for controlling HIV/AIDS. Here we briefly and comprehensively review the essence of contemporary understanding of the test-and-treat policy through mathematical modeling approaches and identify key pitfalls that have been identified to date. While the decrease in HIV incidence is achieved with certain coverages of diagnosis, care and continued treatment, HIV prevalence is not necessarily decreased and sometimes the test-and-treat is accompanied by increased long-term cost of antiretroviral therapy (ART). To confront with the complexity of assessment on this policy, the elimination threshold or the effective reproduction number has been proposed for its use in determining the overall success to anticipate the eventual elimination. Since the publication of original model in 2009, key issues of test-and-treat modeling studies have been identified, including theoretical problems surrounding the sexual partnership network, heterogeneities in the transmission dynamics, and realistic issues of achieving and maintaining high treatment coverage in the most hard-to-reach populations. To explicitly design country-specific control policy, quantitative modeling approaches to each single setting with differing epidemiological context would require multi-disciplinary collaborations among clinicians, public health practitioners, laboratory technologists, epidemiologists and mathematical modelers.

Bilateral Radial Agenesis in a Cat Treated with Bilateral Ulnocarpal Arthrodesis.

PubMed

Bezhentseva, Alla; Singh, Harpreet; Boudrieau, Randy J

2018-06-20

 This article describes corrective antebrachiocarpal re-alignment and arthrodesis for bilateral radial hemimelia (radial agenesis) in an 8-month-old domestic short-haired cat.  Bilateral forelimb deformity of ulnocarpal varus with complete luxation and rotation of the antebrachiocarpal joint spaces, and joint contracture, was observed. Several carpal bones and metacarpal bones I and II and their associated phalanges were absent. Abnormal ambulation and weight bearing on the dorsolateral part of the manus were present. The deformities were treated by bilateral distal ulnar ostectomy and ulnocarpal arthrodesis using a 2.0-mm locking compression plate applied with hybrid fixation and allograft.  Successful deformity correction was obtained with subsequent fusion of the antebrachiocarpal joints. No complications were observed. At long-term follow-up (4.75 years), there was good-to-excellent functional result, with approximately 15° internal rotation of the right forelimb manus and shortened stride with slight circumduction and lameness. All implants remained stable and continued bone remodelling was present. The cat was assessed to have good-to-excellent short- and long-term functional results with excellent owner satisfaction.  Treatment of radial agenesis in the cat has previously been limited to conservative management or limb amputation. While there are several reports of corrective limb-sparing procedures used to treat dogs, this is the first report of a cat with successful salvage corrective surgery. Schattauer GmbH Stuttgart.

Predictors of Silicone Tube Intubation Success in Patients with Lacrimal Drainage System Stenosis.

PubMed

Baek, Ji Sun; Lee, Saem; Lee, Jung Hye; Choi, Hye Sun; Jang, Jae Woo; Kim, Sung Joo

2016-06-01

To evaluate prognostic factors affecting silicone tube intubation outcomes in Asian patients with lacrimal drainage system stenosis. A retrospective review was conducted on the medical records of 822 patients (1,118 eyes) who had undergone silicone tube intubation to treat lacrimal drainage system stenosis between January 2011 and December 2012. Patients were divided into two groups: a success group and a failure group. Success was defined as the disappearance of epiphora symptoms, normalization of tear meniscus height, and the easy passage of fluid without resistance on the postoperative syringing test. Patient and ocular parameters were compared between the success and failure groups. A total of 994 eyes of 727 patients were included in analyses. Patients had a mean follow-up period of 34.11 ± 18.70 weeks. Silicone tube intubation was successful in 67.2% of participants. Significant differences between the success and failure groups were found for age (p < 0.001), history of ipsilateral facial palsy (p = 0.028), follow-up period (p < 0.001), and degree of passage on the preoperative syringing test (p = 0.001). Only age (p < 0.001) and degree of passage on the preoperative syringing test (p = 0.002) remained significantly associated with silicone tube intubation success in multivariate analysis. Age was negatively associated with silicone tube intubation success in patients with lacrimal drainage system stenosis. The success rate was higher in patients who showed easy passage of fluid without resistance on the preoperative syringing test. These factors should be considered by surgeons planning silicone tube intubation in patients with lacrimal drainage system stenosis.

Sirolimus, Tacrolimus, and Antithymocyte Globulin in Preventing Graft-Versus-Host Disease in Patients Undergoing a Donor Stem Cell Transplant For Hematological Cancer

ClinicalTrials.gov

2014-09-03

Chronic Myeloproliferative Disorders; Graft Versus Host Disease; Infection; Leukemia; Lymphoma; Multiple Myeloma and Plasma Cell Neoplasm; Myelodysplastic Syndromes; Myelodysplastic/Myeloproliferative Neoplasms; Precancerous Condition; Secondary Myelofibrosis; Small Intestine Cancer

[Relapsing polychondritis. A case report of a patient treated with methotrexate and celecoxib].

PubMed

Cervera Castillo, Hernando; Torres Caballero, Verónica

2005-01-01

Relapsing polychondritis is a rare illness in which the cartilaginous tissues such as auricles, nose, laryngotracheal structures, joints and others, are affected. Customary treatment is based on corticosteroids and traditional antiinflammatory agents including aspirin and indomethacin. We describe a case of relapsing polychondritis in an 82-year-old man with associated diabetes mellitus and special features treated successfully with alternative therapy based on methotrexate and celecoxib.

Metachronous Medulloblastoma in a Child With Successfully Treated Neuroblastoma: Case Report and Novel Findings of DNA Sequencing.

PubMed

Eterovic, Agda Karina; Maher, Ossama M; Chandra, Joya; Chen, Ken; Huse, Jason; Zaky, Wafik

2018-06-01

Metachronous neoplasms have rarely been reported in patients with neuroblastoma. This report presents the clinical case of a 23-month-old child who was diagnosed with an anaplastic medulloblastoma 5 months after completing treatment for stage IV neuroblastoma. The patient was treated with complete surgical resection and adjuvant chemoradiation followed by maintenance chemotherapy at an outside institution and came to our institution for further management. A pathologic diagnosis and review of both the suprarenal and posterior fossa masses were performed, as well as a genetic analysis of both cerebellar tumor tissue and blood using next-generation gene sequencing. At our institution, the patient was submitted to induction chemotherapy followed by high-dose chemotherapy and autologous stem cell transplantation and remains free of disease 2 years after completion of treatment. Genetic analysis revealed multiple somatic copy number variations with most deleted genes located in 2q37, a region which harbors genes involved in epigenetic regulation and tumor suppression. A homozygous deletion was found in the TSC2 gene, which is a clinically actionable gene, and patients with activating deletions in TSC2 can potentially be eligible for basket clinical trials with mTOR inhibitors. Germline single nucleotide variants were also identified in multiple genes involved in cancer (ALK, FGFR3, FLT3/4, HNF1A, NCOR1 , and NOTCH2/3 ), cancer predisposition ( TP53, TSC1 , and BRCA1/2) , and genes involved in DNA repair ( MSH6, PMS2, POLE , and ATM ). Metachronous neoplasms are rare and challenging to treat, hence genetic analysis and referral are needed to exclude hereditary cause. DNA sequencing of the tumor and germline can help identify alterations that increase predisposition or can be used to guide treatment decisions on recurrence and when standard options fail. Copyright © 2018 by the National Comprehensive Cancer Network.

The Peru Cervical Cancer Screening Study (PERCAPS): The Design and Implementation of a Mother/Daughter Screen, Treat, and Vaccinate Program in the Peruvian Jungle

PubMed Central

Levinson, Kimberly L.; Salmeron, Jorge; Sologuren, Carlos Vallejos; Fernandez, Maria Jose Vallejos; Belinson, Jerome L.

2014-01-01

Peru struggles to prevent cervical cancer (CC). In the jungle, prevention programs suffer from significant barriers although technology exists to detect CC precursors. This study used community based participatory research (CBPR) methods to overcome barriers. The objective was to evaluate the utility of CBPR techniques in a mother–child screen/treat and vaccinate program for CC prevention in the Peruvian jungle. The CC prevention program used self-sampling for human papillomavirus (HPV) for screening, cryotherapy for treatment and the HPV vaccine Gardasil for vaccination. Community health leaders (HL) from around Iquitos participated in a two half day educational course. The HLs then decided how to implement interventions in their villages or urban sectors. The success of the program was measured by: (1) ability of the HLs to determine an implementation plan, (2) proper use of research forms, (3) participation and retention rates, and (4) participants’ satisfaction. HLs successfully registered 320 women at soup kitchens, schools, and health posts. Screening, treatment, and vaccination were successfully carried out using forms for registration, consent, and results with minimum error. In the screen/treat intervention 100 % of participants gave an HPV sample and 99.7 % reported high satisfaction; 81 % of HPV + women were treated, and 57 % returned for 6-month followup. Vaccine intervention: 98 % of girls received the 1st vaccine, 88 % of those received the 2nd, and 65 % the 3rd. CBPR techniques successfully helped implement a screen/treat and vaccinate CC prevention program around Iquitos, Peru. These techniques may be appropriate for large-scale preventive health-care interventions. PMID:24276617

The Peru Cervical Cancer Screening Study (PERCAPS): the design and implementation of a mother/daughter screen, treat, and vaccinate program in the Peruvian jungle.

PubMed

Abuelo, Carolina E; Levinson, Kimberly L; Salmeron, Jorge; Sologuren, Carlos Vallejos; Fernandez, Maria Jose Vallejos; Belinson, Jerome L

2014-06-01

Peru struggles to prevent cervical cancer (CC). In the jungle, prevention programs suffer from significant barriers although technology exists to detect CC precursors. This study used community based participatory research (CBPR) methods to overcome barriers. The objective was to evaluate the utility of CBPR techniques in a mother-child screen/treat and vaccinate program for CC prevention in the Peruvian jungle. The CC prevention program used self-sampling for human papillomavirus (HPV) for screening, cryotherapy for treatment and the HPV vaccine Gardasil for vaccination. Community health leaders (HL) from around Iquitos participated in a two half day educational course. The HLs then decided how to implement interventions in their villages or urban sectors. The success of the program was measured by: (1) ability of the HLs to determine an implementation plan, (2) proper use of research forms, (3) participation and retention rates, and (4) participants' satisfaction. HLs successfully registered 320 women at soup kitchens, schools, and health posts. Screening, treatment, and vaccination were successfully carried out using forms for registration, consent, and results with minimum error. In the screen/treat intervention 100% of participants gave an HPV sample and 99.7% reported high satisfaction; 81% of HPV + women were treated, and 57% returned for 6-month followup. Vaccine intervention: 98% of girls received the 1st vaccine, 88% of those received the 2nd, and 65% the 3rd. CBPR techniques successfully helped implement a screen/treat and vaccinate CC prevention program around Iquitos, Peru. These techniques may be appropriate for large-scale preventive health-care interventions.

Infection Prophylaxis and Management in Treating Cytomegalovirus (CMV) Infection in Patients With Hematologic Malignancies Previously Treated With Donor Stem Cell Transplant

ClinicalTrials.gov

2015-06-03

; Noncontiguous Stage II Adult Burkitt Lymphoma; Noncontiguous Stage II Adult Diffuse Large Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Mixed Cell Lymphoma; Noncontiguous Stage II Adult Diffuse Small Cleaved Cell Lymphoma; Noncontiguous Stage II Adult Immunoblastic Large Cell Lymphoma; Noncontiguous Stage II Adult Lymphoblastic Lymphoma; Noncontiguous Stage II Grade 1 Follicular Lymphoma; Noncontiguous Stage II Grade 2 Follicular Lymphoma; Noncontiguous Stage II Grade 3 Follicular Lymphoma; Noncontiguous Stage II Mantle Cell Lymphoma; Noncontiguous Stage II Marginal Zone Lymphoma; Noncontiguous Stage II Small Lymphocytic Lymphoma; Polycythemia Vera; Post-transplant Lymphoproliferative Disorder; Previously Treated Myelodysplastic Syndromes; Primary Myelofibrosis; Primary Systemic Amyloidosis; Progressive Hairy Cell Leukemia, Initial Treatment; Prolymphocytic Leukemia; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Refractory Multiple Myeloma; Relapsing Chronic Myelogenous Leukemia; Secondary Acute Myeloid Leukemia; Secondary Myelodysplastic Syndromes; Secondary Myelofibrosis; Splenic Marginal Zone Lymphoma; Stage 0 Chronic Lymphocytic Leukemia; Stage I Adult

Keratomycosis due to Tintelnotia destructans refractory to common therapy treated successfully with systemic and local terbinafine in combination with polyhexamethylene biguanide.

PubMed

Behrens-Baumann, Wolfgang J; Hofmüller, Wolfram; Tammer, Ina; Tintelnot, Kathrin

2018-04-28

To report on a wearer of rigid gas-permeable contact lenses with a keratomycosis due to Tintelnotia-a new genus of Phaeosphaeriaceae-treated with terbinafine and polyhexamethylene biguanide. Chart review of a patient with fungal keratitis treated additionally with systemic and topical terbinafine 0.25% after symptoms increased under conventional antimycotic therapy with voriconazole. Antifungal susceptibility had been tested in vitro. After starting an additional treatment with systemic and topical terbinafine, the severe corneal infection was sufficiently resolved. The drug was well tolerated without any neurological, dermatological or gastroenterological problems. Terbinafine revealed a marked in vitro antifungal activity of 0.12 µg/ml. The fungus was identified as Tintelnotia destructans. Terbinafine might be considered as a therapeutic option in severe cases of fungal keratitis refractory to common antifungal therapy.

Successful leukocytapheresis therapy in a patient with rheumatoid arthritis on maintenance hemodialysis.

PubMed

Maeshima, Keisuke; Torigoe, Masataka; Iwakura, Mikako; Yamanaka, Kunitoshi; Ishii, Koji

2015-01-01

We report the case of a 44-year-old female undergoing maintenance hemodialysis in whom early-phase rheumatoid arthritis (RA) was successfully treated by leukocytapheresis (LCAP). The effects of prednisone, tacrolimus, and etanercept were limited, but LCAP was highly effective and its efficacy continued even after cessation of LCAP. Moreover, remission was maintained for 2 years after discontinuation of medication. LCAP may be an important treatment option for RA patients with end-stage renal failure who are on hemodialysis.

TREAT Neutronics Analysis of Water-Loop Concept Accommodating LWR 9-rod Bundle

DOE Office of Scientific and Technical Information (OSTI.GOV)

Hill, Connie M.; Woolstenhulme, Nicolas E.; Parry, James R.

Abstract. Simulation of a variety of transient conditions has been successfully achieved in the Transient Reactor Test (TREAT) facility during operation between 1959 and 1994 to support characterization and safety analysis of nuclear fuels and materials. A majority of previously conducted tests were focused on supporting sodium-cooled fast reactor (SFR) designs. Experiments evolved in complexity. Simulation of thermal-hydraulic conditions expected to be encountered by fuels and materials in a reactor environment was realized in the development of TREAT sodium loop experiment vehicles. These loops accommodated up to 7-pin fuel bundles and served to simulate more closely the reactor environment whilemore » safely delivering large quantities of energy into the test specimen. Some of the immediate TREAT restart operations will be focused on testing light water reactor (LWR) accident tolerant fuels (ATF). Similar to the sodium loop objectives, a water loop concept, developed and analyzed in the 1990’s, aimed at achieving thermal-hydraulic conditions encountered in commercial power reactors. The historic water loop concept has been analyzed in the context of a reactivity insertion accident (RIA) simulation for high burnup LWR 2-pin and 3-pin fuel bundles. Findings showed sufficient energy could be deposited into the specimens for evaluation. Similar results of experimental feasibility for the water loop concept (past and present) have recently been obtained using MCNP6.1 with ENDF/B-VII.1 nuclear data libraries. The old water loop concept required only two central TREAT core grid spaces. Preparation for future experiments has resulted in a modified water loop conceptual design designated the TREAT water environment recirculating loop (TWERL). The current TWERL design requires nine TREAT core grid spaces in order to place the water recirculating pump under the TREAT core. Due to the effectiveness of water moderation, neutronics analysis shows that removal of seven

Perceived neighborhood safety, recovery capital, and successful outcomes among mothers 10 years after substance abuse treatment

PubMed Central

Evans, E.; Li, L.; Buoncristiani, S.; Hser, Y.I.

2014-01-01

This study examines perceived neighborhood characteristics associated with successful outcome among mothers 10 years after being treated for substance use disorders. Data were obtained from 713 mothers first studied at admission to drug treatment in California in 2000-2002 and followed-up in 2009-2011. At follow-up, 53.6% of mothers had a successful outcome (i.e., no use of illicit drugs and not involved with the criminal justice system). Perceived neighborhood safety almost doubled the odds of success. Perceived neighborhood safety interacted with social involvement, decreasing the odds of success among mothers who reported more versus less neighborhood social involvement. Perceived neighborhood climate is associated with long-term outcomes among mothers with substance use disorders independent of individual-level characteristics, underscoring the need for further efforts to understand its interaction with recovery capital in ways that promote and impede health. PMID:24832914

Successful salvage treatment of native valve Enterococcus faecalis infective endocarditis with telavancin: two case reports.

PubMed

Thompson, Mickala M; Hassoun, Ali

2017-07-01

Infective endocarditis (IE) one-year mortality rates approach 40%. Here, we report two native valve Enterococcus faecalis IE cases in patients successfully treated with telavancin. An 88-year-old with mitral valve endocarditis and a penicillin allergy, initially treated with intravenous vancomycin, was switched to telavancin. A 69-year-old, who previously received amoxicillin and intravenous vancomycin for presumed enterococcal bacteraemia, was diagnosed with dual valve endocarditis for which he received telavancin. Both received six weeks of telavancin. Neither had telavancin-related adverse events, evidence of infection at six months, nor required telavancin dosing adjustments. Documented use of novel treatments for serious enterococcal infections is needed.

Successful Coil Embolization of a Ruptured Basilar Artery Aneurysm in a Child with Leukemia: A Case Report

PubMed Central

HAYASHI, Shihori; MAEHARA, Taketoshi; MUKAWA, Maki; AOYAGI, Masaru; YOSHINO, Yoshikazu; NEMOTO, Shigeru; ONO, Toshiaki; OHNO, Kikuo

2014-01-01

Ruptured intracranial aneurysms are rare in the pediatric population compared to adults. This has incited considerable discussion on how to treat children with this condition. Here, we report a child with a ruptured saccular basilar artery aneurysm that was successfully treated with coil embolization. A 12-year-old boy with acute lymphoblastic leukemia and accompanying abdominal candidiasis after chemotherapy suddenly complained of a severe headache and suffered consciousness disturbance moments later. Computed tomography scans and cerebral angiography demonstrated acute hydrocephalus and subarachnoid hemorrhage caused by saccular basilar artery aneurysm rupture. External ventricular drainage was performed immediately. Because the patient was in severe condition and did not show remarkable signs of central nervous system infection in cerebrospinal fluid studies, we applied endovascular treatment for the ruptured saccular basilar artery aneurysm, which was successfully occluded with coils. The patient recovered without new neurological deficits after ventriculoperitoneal shunting. Recent reports indicate that both endovascular and microsurgical techniques can be used to effectively treat ruptured cerebral aneurysms in pediatric patients. A minimally invasive endovascular treatment was effective in the present case, but long-term follow-up will be necessary to confirm the efficiency of endovascular treatment for children with ruptured saccular basilar artery aneurysms. PMID:24257487

Successful coil embolization of a ruptured basilar artery aneurysm in a child with leukemia: a case report.

PubMed

Hayashi, Shihori; Maehara, Taketoshi; Mukawa, Maki; Aoyagi, Masaru; Yoshino, Yoshikazu; Nemoto, Shigeru; Ono, Toshiaki; Ohno, Kikuo

2014-01-01

Ruptured intracranial aneurysms are rare in the pediatric population compared to adults. This has incited considerable discussion on how to treat children with this condition. Here, we report a child with a ruptured saccular basilar artery aneurysm that was successfully treated with coil embolization. A 12-year-old boy with acute lymphoblastic leukemia and accompanying abdominal candidiasis after chemotherapy suddenly complained of a severe headache and suffered consciousness disturbance moments later. Computed tomography scans and cerebral angiography demonstrated acute hydrocephalus and subarachnoid hemorrhage caused by saccular basilar artery aneurysm rupture. External ventricular drainage was performed immediately. Because the patient was in severe condition and did not show remarkable signs of central nervous system infection in cerebrospinal fluid studies, we applied endovascular treatment for the ruptured saccular basilar artery aneurysm, which was successfully occluded with coils. The patient recovered without new neurological deficits after ventriculoperitoneal shunting. Recent reports indicate that both endovascular and microsurgical techniques can be used to effectively treat ruptured cerebral aneurysms in pediatric patients. A minimally invasive endovascular treatment was effective in the present case, but long-term follow-up will be necessary to confirm the efficiency of endovascular treatment for children with ruptured saccular basilar artery aneurysms.

Long-Term Prophylactic Antibiotic Treatment: Effects on Survival, Immunocompetence and Reproduction Success of Parasemia plantaginis (Lepidoptera: Erebidae).

PubMed

Dickel, Franziska; Freitak, Dalial; Mappes, Johanna

2016-01-01

Hundreds of insect species are nowadays reared under laboratory conditions. Rearing of insects always implicates the risk of diseases, among which microbial infections are the most frequent and difficult problems. Although there are effective prophylactic treatments, the side effects of applied antibiotics are not well understood. We examined the effect of prophylactic antibiotic treatment on the overwintering success of wood tiger moth (Parasemia plantaginis) larvae, and the postdiapause effect on their life-history traits. Four weeks before hibernation larvae were treated with a widely used antibiotic (fumagillin). We monitored moths' survival and life-history traits during the following 10 mo, and compared them to those of untreated control larvae. Prophylactic antibiotic treatment had no effect on survival but we show effects on some life-history traits by decreasing the developmental time of treated larvae. However, we also revealed relevant negative effects, as antibiotic treated individuals show a decreased number of laid eggs and also furthermore a suppressed immunocompetence. These results implicate, that a prophylactic medication can also lead to negative effects on life-history traits and reproductive success, which should be seriously taken in consideration when applying a prophylactic treatment to laboratory reared insect populations. © The Author 2016. Published by Oxford University Press on behalf of the Entomological Society of America.

I-95 phobia treated with hypnotic systematic desensitization: a case report.

PubMed

Iglesias, Alex; Iglesias, Alex; Iglesias, Adam

2013-10-01

Systematic desensitization and hypnosis mediated therapy share empirical evidence of efficacy in the treatment of specific phobias. However, a review of the literature indicated there is limited documentation in the employment of these modalities for treating driving related phobias (DRP). This article reports on the use of hypnosis aided systematic desensitization (HASD) in the successful treatment of a case of non-accident related driving phobia, specifically manifested on Interstate 95 (I-95). The treatment consisted of 6 office sessions of HASD along with 14 in-vivo sessions where the patient performed multiple exposures/rehearsals of the behaviors that had been successfully mastered at the office visits. The results indicated that this patient with case of (DRP) was able to resume travel on I-95 at conclusion of treatment. The patient was symptom free at follow up 6 months later.

Dynamic Leadership Succession: Strengthening Urban Principal Succession Planning

ERIC Educational Resources Information Center

Peters-Hawkins, April L.; Reed, Latish C.; Kingsberry, Francemise

2018-01-01

The Dynamic Leadership Succession model is used to analyze a leadership succession case in an urban school district. The qualitative findings show that the district did not forecast school leadership needs well; however, the principal sought to develop and mentor teacher leaders as her assistant principals. Second, sustaining efforts within the…

Successful Renal Transplantation with Desensitization in Highly Sensitized Patients: A Single Center Experience

PubMed Central

Yoon, Hye Eun; Hyoung, Bok Jin; Hwang, Hyeon Seok; Lee, So Young; Jeon, Youn Joo; Song, Joon Chang; Oh, Eun-Jee; Park, Sun Cheol; Choi, Bum Soon; Moon, In Sung; Kim, Yong Soo

2009-01-01

Intravenous immunoglobulin (IVIG) and/or plasmapheresis (PP) are effective in preventing antibody-mediated rejection (AMR) of kidney allografts, but AMR is still a problem. This study reports our experience in living donor renal transplantation in highly sensitized patients. Ten patients with positive crossmatch tests or high levels of panel-reactive antibody (PRA) were included. Eight patients were desensitized with pretransplant PP and low dose IVIG, and two were additionally treated with rituximab. Allograft function, number of acute rejection (AR) episodes, protocol biopsy findings, and the presence of donor-specific antibody (DSA) were evaluated. With PP/IVIG, six out of eight patients showed good graft function without AR episodes. Protocol biopsies revealed no evidence of tissue injury or C4d deposits. Of two patients with AR, one was successfully treated with PP/IVIG, but the other lost graft function due to de novo production of DSA. Thereafter, rituximab was added to PP/IVIG in two cases. Rituximab gradually decreased PRA levels and the percentage of peripheral CD20+ cells. DSA was undetectable and protocol biopsy showed no C4d deposits. The graft function was stable and there were no AR episodes. Conclusively, desensitization using PP/IVIG with or without rituximab increases the likelihood of successful living donor renal transplantation in sensitized recipients. PMID:19194545

Sunitinib Malate in Treating HIV-Positive Patients With Cancer Receiving Antiretroviral Therapy

ClinicalTrials.gov

2014-03-14

-transplant Lymphoproliferative Disorder; Previously Treated Myelodysplastic Syndromes; Primary Myelofibrosis; Primary Systemic Amyloidosis; Progressive Hairy Cell Leukemia, Initial Treatment; Prolymphocytic Leukemia; Recurrent Adult Acute Lymphoblastic Leukemia; Recurrent Adult Acute Myeloid Leukemia; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Grade III Lymphomatoid Granulomatosis; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Renal Cell Cancer; Recurrent Small Lymphocytic Lymphoma; Refractory Chronic Lymphocytic Leukemia; Refractory Hairy Cell Leukemia; Refractory Multiple Myeloma; Relapsing Chronic Myelogenous Leukemia; Stage IV Renal Cell Cancer; T-cell Large Granular Lymphocyte Leukemia; Testicular Lymphoma; Unspecified Adult Solid Tumor, Protocol Specific; Waldenström Macroglobulinemia

A complete investigation of monocular and binocular functions in clinically treated amblyopia.

PubMed

Zhao, Wuxiao; Jia, Wu-Li; Chen, Ge; Luo, Yan; Lin, Borong; He, Qing; Lu, Zhong-Lin; Li, Min; Huang, Chang-Bing

2017-09-06

The gold standard of a successful amblyopia treatment is full recovery of visual acuity (VA) in the amblyopic eye, but there has been no systematic study on both monocular and binocular visual functions. In this research, we aimed to quantify visual qualities with a variety of perceptual tasks in subjects with treated amblyopia. We found near stereoacuity and pAE dominance in binocular rivalry in "treated" amblyopia were largely comparable to those of normal subjects. CSF of the pAE remained deficient in high spatial frequencies. The binocular contrast summation ratio is significantly lower than normal standard. The interocular balance point is 34%, indicating that contrast in pAE is much less effective as the same contrast in pFE in binocular phase combination. Although VA, stereoacuity and binocular rivalry at low spatial frequency in treated amblyopes were normal or nearly normal, the pAE remained "lazy" in high frequency domain, binocular contrast summation, and interocular phase combination. Our results suggest that structured monocular and binocular training are necessary to fully recover deficient functions in amblyopia.

Parametric Characterization of Flow Inside Cererbal Aneurysms Treated with Flow-Diverting Stents

NASA Astrophysics Data System (ADS)

Barbour, Michael; Levitt, Michael; Geindreau, Christian; Johnson, Luke; Chivukula, Keshav; Aliseda, Alberto

2017-11-01

Cerebral aneurysms are often treated with a flow-diverting stent (FDS) to reduce blood flow into the aneurysm sac, promoting the development of a stable thrombus. Successful treatment is highly dependent on the degree of flow reduction and the altered hemodynamics inside the aneurysm sac following treatment. Establishing a causal connection between hemodynamic metrics of FDS-treated CAs and long-term clinical outcomes requires a rigorous parametric characterization of this flow environment. We use 3D particle image velocimetry (PIV) to measure the flow inside idealized aneurysm models treated with FDS. Physiologically realistic Reynolds numbers and increasing levels of parent vessel curvature are analyzed to understand the effect of inertia on flow development. The flow velocity into the aneurysm and the topology of the flow inside the sac is shown to be highly dependent on parent vessel Dean number (De). The role of flow pulsatility is then added to the study via time-dependent waveforms. Velocity measurements at 2 values of parent vessel Womersley number (Wo) allow us to parameterize flow inside of CAs treated with FDS as a function of De, Re and Wo, improving the fundamental understanding of how FDS alter CA hemodynamics and aiding in the development of new treatments.

Successful treatment of Beauveria bassiana fungal keratitis with topical voriconazole.

PubMed

Ogawa, Akiko; Matsumoto, Yukihiro; Yaguchi, Takashi; Shimmura, Shigeto; Tsubota, Kazuo

2016-04-01

We describe a 66-year-old woman who suffered from fungal keratitis after corneal transplantation. The causative organism was identified as Beauveria bassiana on the basis of morphological characteristics and the sequence of the internal transcribed spacer region of the ribosomal RNA gene. The patient was successfully treated with topical voriconazole (VRCZ) use only. We, hereby, present the first report of a case with B. bassiana fungal keratitis that responded to topical antifungal VRCZ treatment. Copyright © 2015 Japanese Society of Chemotherapy and The Japanese Association for Infectious Diseases. Published by Elsevier Ltd. All rights reserved.

Comparative study between botulinum toxin injection and partial division of puborectalis for treating anismus.

PubMed

Farid, Mohamed; Youssef, Tamer; Mahdy, Tarek; Omar, Waleed; Moneim, Hesham Abdul; El Nakeeb, Ayman; Youssef, Mohamed

2009-03-01

The objective of this study was to compare the results of partial division of puborectalis (PDPR) versus local botulinum toxin type A (BTX-A) injection in treating patients with anismus. This prospective randomized study included 30 male patients suffering from anismus. Diagnosis was made by clinical examination, barium enema, colonoscopy, colonic transit time, anorectal manometry, balloon expulsion test, defecography, and electromyography. Patients were randomized into: group I which included 15 patients who were injected with BTX-A and group II which included 15 patients who underwent bilateral PDPR. Follow-up was conducted for about 1 year. Improvement was considered when patients returned to their normal habits. BTX-A injection achieved initial success in 13 patients (86.7%). However, long-term success persisted only in six patients (40%). This was in contrast to PDPR which achieved initial success in all patients (100%) with a long-term success in ten patients (66.6%). Recurrence was observed in seven patients (53.8%) and five patients (33.4%) following BTX-A injection and PDPR, respectively. Minor degrees of incontinence were confronted in two patients (13.3%) following PDPR. BTX-A injection seems to be successful for temporary treatment of anismus.

Fertility in patients treated for testicular cancer.

PubMed

Matos, Erika; Skrbinc, Breda; Zakotnik, Branko

2010-09-01

Testicular cancer affects men mostly in their reproductive age with a cure rate over 90% and fertility is one of the main concerns of survivors. To further elucidate the question of fertility after treatment for testicular cancer, we performed a survey in patients treated in our institution. We sent a questionnaire to patients treated for testicular cancer at our institute from 1976 to 2002 (n = 490) of whom 297 (60.6%) responded. We considered the patients to have conserved fertility if they had children after treatment without assisted reproductive technologies. Before treatment 119/297 (40.1%) of patients and after treatment 150/297 (50.5%) of patients tried to have children (p = 0.019). Of 119 patients who tried to have children before treatment for testicular cancer 98 (82.4%) succeeded and 74/150 (49.3%) were successful after treatment (p < 0.001). After treatment patients had 1-3 (median 1) children. The median time to birth of first child from diagnosis was 12 years. The post-treatment fatherhood in patients treated with surgery only (orchidectomy +/- retroperitoneal lymphnode dissection-RPLND) was 59%, in those with additional radiotherapy 68%, and chemotherapy 50% (p = 0.233). Fertility rate in patients where a non nerve sparing RPLND was performed was only 37%, 62% in patients with nerve sapring RPLND, and 77% in patients where RPLND was not performed (p < 0.0001). Fertility rate after treatment for testicular cancer is reduced. From our data, the most important treatment modality that influences fertility is non nerve sparing RPLND that should be avoided whenever possible in order improve the quality of life our patients.

Myeloproliferative Neoplasms (MPNs) Patient Registry

ClinicalTrials.gov

2017-10-27

Primary Myelofibrosis; Polycythemia Vera; Essential Thrombocythemia; Mastocytosis; Leukemia, Myeloid, Chronic, Atypical, BCR-ABL Negative; Leukemia, Myelomonocytic, Juvenile; Chronic Eosinophilic Leukemia-not Otherwise Specified; Myelodysplastic-Myeloproliferative Diseases; Neoplasms; Leukemia, Myelomonocytic, Chronic

Inter-Disciplinary Collaboration in Support of the Post-Standby TREAT Mission

DOE Office of Scientific and Technical Information (OSTI.GOV)

DeHart, Mark; Baker, Benjamin; Ortensi, Javier

Although analysis methods have advanced significantly in the last two decades, high fidelity multi- physics methods for reactors systems have been under development for only a few years and are not presently mature nor deployed. Furthermore, very few methods provide the ability to simulate rapid transients in three dimensions. Data for validation of advanced time-dependent multi- physics is sparse; at TREAT, historical data were not collected for the purpose of validating three-dimensional methods, let alone multi-physics simulations. Existing data continues to be collected to attempt to simulate the behavior of experiments and calibration transients, but it will be insufficient formore » the complete validation of analysis methods used for TREAT transient simulations. Hence, a 2018 restart will most likely occur without the direct application of advanced modeling and simulation methods. At present, the current INL modeling and simulation team plans to work with TREAT operations staff in performing reactor simulations with MAMMOTH, in parallel with the software packages currently being used in preparation for core restart (e.g., MCNP5, RELAP5, ABAQUS). The TREAT team has also requested specific measurements to be performed during startup testing, currently scheduled to run from February to August of 2018. These startup measurements will be crucial in validating the new analysis methods in preparation for ultimate application for TREAT operations and experiment design. This document describes the collaboration between modeling and simulation staff and restart, operations, instrumentation and experiment development teams to be able to effectively interact and achieve successful validation work during restart testing.« less

Cystic chondromalacia of the auricle treated with dual-plane excision with intracartilaginous dissection.

PubMed

Zoccali, Giovanni; Pajand, Reza; Vrentzos, Nikolaos; Giuliani, Maurizio

2014-09-01

Cystic chondromalacia of the auricle is an uncommon condition in which a degenerative process occurs within the cartilage. The disorder affects young and middle-aged people. Clinically, it manifests as a painless, fluctuant swelling that frequently relapses despite various therapeutic approaches. In this article we report a typical case of cystic chondromalacia of the auricle that was successfully treated by surgery-specifically, dual-plane dissection-and we briefly review the literature.

Acute colonic pseudoobstruction in a child with sickle cell disease treated with neostigmine.

PubMed

Khosla, Arjun; Ponsky, Todd A

2008-12-01

Sickle cell disease is a disorder that produces significant morbidity and mortality. Vaso-occlusive pain crises are the most common presenting symptom associated with sickle cell patients. A rare, yet important to recognize, complication of sickle cell disease is acute colonic pseudoobstruction, also known as Ogilvie's syndrome. These patients may present with symptoms that are difficult to distinguish from other etiologies of abdominal pain, but a thorough diagnostic workup can provide important clues. Furthermore, there is no agreement on optimal treatment of pseudoobstruction. We report the first pediatric case of acute pseudoobstruction secondary to sickle cell disease that was treated successfully with neostigmine. Early recognition of this phenomenon is important as it alters patient management, can be treated medically, and may avoid unnecessary surgical intervention.

Optogenetic approaches to treat epilepsy.

PubMed

Wykes, Robert C; Kullmann, Dimitri M; Pavlov, Ivan; Magloire, Vincent

2016-02-15

Novel treatments for drug-resistant epilepsy are required. Optogenetics is a combination of optical and genetic methods used to control the activity of specific populations of excitable cells using light with high temporal and spatial resolution. Derived from microbial organisms, 'opsin' genes encode light-activated ion channels and pumps. Opsins can be genetically targeted to well-defined neuronal populations in mammalian brains using viral vectors. When exposed to light of an appropriate wavelength, the excitability of neurons can be increased or decreased optically on a millisecond timescale. Alternative treatments for drug-resistant epilepsy such as vagal, cortical or subcortical stimulation, focal cooling, callosotomy, or ketogenic diet have met with limited success, whereas optogenetic approaches have shown considerable pre-clinical promise. Several groups have reported that optogenetic approaches successfully attenuated epileptiform activity in different rodent models of epilepsy, providing proof of the principle that this approach may translate to an effective treatment for epilepsy patients. However, further studies are required to determine the optimal opsin, in which types (or subtypes) of neurons it should be expressed, and what are the most efficient temporal profiles of photostimulation. Although invasive due to the need to inject a viral vector into the brain and implant a device to deliver light to opsin-transduced neurons, this approach has the potential to be effective in suppressing spontaneous seizures while avoiding the side-effects of anti-epileptic drugs (AEDs) or the need to permanently excise regions of the brain. Optogenetic approaches may treat drug-refractory epilepsies. Copyright © 2015 Elsevier B.V. All rights reserved.

A Case of Chronic Total Occlusion of the Left Anterior Descending Artery Successfully Treated with Side Branch Technique Using the Soutenir CV

PubMed Central

Niizeki, Takeshi; Ikeno, Eiichiro; Kubota, Isao

2017-01-01

Patient: Male, 54 Final Diagnosis: Old myocardial infarction Symptoms: Lower extremity swelling • respiratory distress Medication: — Clinical Procedure: Success Specialty: Cardiology Objective: Unusual setting of medical care Background: Success rates for treatment of chronic total occlusion (CTO) have dramatically improved in recent years with the development of new CTO guidewires and development of new techniques such as the retrograde approach. In the antegrade approach, a guidewire is occasionally passed through a side branch despite successful wire crossing of the CTO lesion. In order to pass a wire through the main artery, there are a few side branch techniques such as a reverse wire technique. Case Report: A 54-year-old man with symptoms of heart failure was admitted to our hospital. Coronary angiography showed CTO of the proximal left anterior descending artery. Percutaneous coronary intervention with an antegrade approach was started. We succeeded in passing the wire through a side branch but not the main artery. Unfortunately, a reverse wire technique failed in this case. Next, the wire passed through a side branch was exchanged with the Soutenir CV, and a retrograde approach was started. The wire crossing from retrograde was entwined around the Soutenir CV. After that, the retrograde wire was snared and guided to the antegrade guiding catheter, which resulted in successful wiring into the main artery easily. Conclusions: The side branch technique using the Soutenir CV may be an effective strategy in some cases. PMID:28082733

Polycystic Ovarian Syndrome-associated Confluent and Reticulated Papillomatosis: Report of a Patient Successfully Treated with Azithromycin.

PubMed

Fite, Laura Paul; Cohen, Philip R

2017-09-01

Polycystic ovarian syndrome is a common endocrine disorder with a variety of dermatologic manifestations among young women. Confluent and reticulated papillomatosis is a rare dermatosis of unknown etiology that is seldom reported in patients with polycystic ovarian syndrome. We describe the case of a young woman with obesity, confluent and reticulated papillomatosis, and concurrent acanthosis nigricans. Her history, physical examination, and laboratory evaluation led to the diagnosis of polycystic ovarian syndrome. The proposed etiologies and the various of treatment options for confluent and reticulated papillomatosis are discussed. In our case, the patient had a dramatic response to treatment with azithromycin. The etiology of confluent and reticulated papillomatosis remains to be established. Additionally, the mechanism behind the success of treatment with antibiotics is unclear; however, in this patient, azithromycin was a safe and effective option for the treatment of confluent and reticulated papillomatosis.

Genetics Home Reference: primary myelofibrosis

MedlinePlus

... from gene mutations that occur in early blood-forming cells after conception. These alterations are called somatic ... Free article on PubMed Central Klampfl T, Gisslinger H, Harutyunyan AS, Nivarthi H, Rumi E, Milosevic JD, ...

Azadirachtin effects on mating success, gametic abnormalities and progeny survival in Drosophila melanogaster (Diptera).

PubMed

Oulhaci, Chemseddine M; Denis, Béatrice; Kilani-Morakchi, Samira; Sandoz, Jean-Christophe; Kaiser, Laure; Joly, Dominique; Aribi, Nadia

2018-01-01

Azadirachtin is a prominent natural pesticide and represents an alternative to conventional insecticides. It has been successfully used against insect pests. However, its effects on reproduction require further analysis. Here we investigated lethal and sublethal effects of azadirachtin, on treated adults in a model insect, Drosophila melanogaster (Meigen). Dose-mortality relationships as well as several parameters of reproduction (mating, spermatogenesis, oogenesis and fertility) were examined. Neem-Azal, a commercial formulation of azadirachtin, applied topically on newly emerged adults, increased mortality with a positive dose-dependent relationship. The LD 50 (0.63 μg) was determined 24 h after treatment using a non-linear regression. Adults surviving this dose had a mating success that was divided by 3 and a progeny production reduced by half when males were treated, and even more when females were treated. When combining probability of survival, of mating and reduced progeny, it appeared that LD 50 induced a 98% reduction in reproductive rates. Reduced progeny was partially explained by the effect of adult treatment on gametes number and abnormalities. The number of cysts and the apical nuclei positions within the cysts decreased by 29.7% and 20%, respectively, in males. In females, the number of oocytes per ovary and the volume of basal oocytes also decreased by 16.1% and 32.4%, respectively. Azadirachtin causes significant toxic effects in both sexes and decreases the fecundity and fertility of D. melanogaster. Females are more sensitive to azadirachtin. © 2017 Society of Chemical Industry. © 2017 Society of Chemical Industry.

Successful Treatment of Passive Fecal Incontinence in an Animal Model Using Engineered Biosphincters: A 3‐Month Follow‐Up Study

PubMed Central

Bohl, Jaime L.; Zakhem, Elie

2017-01-01

Abstract Fecal incontinence (FI) is the involuntary passage of fecal material. Current treatments have limited successful outcomes. The objective of this study was to develop a large animal model of passive FI and to demonstrate sustained restoration of fecal continence using anorectal manometry in this model after implantation of engineered autologous internal anal sphincter (IAS) biosphincters. Twenty female rabbits were used in this study. The animals were divided into three groups: (a) Non‐treated group: Rabbits underwent IAS injury by hemi‐sphincterectomy without treatment. (b) Treated group: Rabbits underwent IAS injury by hemi‐sphincterectomy followed by implantation of autologous biosphincters. (c) Sham group: Rabbits underwent IAS injury by hemi‐sphincterectomy followed by re‐accessing the surgical site followed by immediate closure without implantation of biosphincters. Anorectal manometry was used to measure resting anal pressure and recto‐anal inhibitory reflex (RAIR) at baseline, 1 month post‐sphincterectomy, up to 3 months after implantation and post‐sham. Following sphincterectomy, all rabbits had decreased basal tone and loss of RAIR, indicative of FI. Anal hygiene was also lost in the rabbits. Decreases in basal tone and RAIR were sustained more than 3 months in the non‐treated group. Autologous biosphincters were successfully implanted into eight donor rabbits in the treated group. Basal tone and RAIR were restored at 3 months following biosphincter implantation and were significantly higher compared with rabbits in the non‐treated and sham groups. Histologically, smooth muscle reconstruction and continuity was restored in the treated group compared with the non‐treated group. Results in this study provided promising outcomes for treatment of FI. Results demonstrated the feasibility of developing and validating a large animal model of passive FI. This study also showed the efficacy of the engineered biosphincters to restore

Autoimmune hepatitis during intravenous glucocorticoid pulse therapy for Graves' ophthalmopathy treated successfully with glucocorticoids themselves.

PubMed

Marinò, M; Morabito, E; Altea, M A; Ambrogini, E; Oliveri, F; Brunetto, M R; Pollina, L E; Campani, D; Vitti, P; Bartalena, L; Pincheral, A; Marcocci, C

2005-03-01

We report a case of acute hepatitis of autoimmune origin which occurred in a 43-yr-old woman during iv glucocorticoid (GC) pulse therapy for Graves' ophthalmopathy (GO). Prior to therapy, liver function tests were normal with no previous history of liver disorders or conditions predisposing to GC-associated liver damage. After the administration of a 4.7-g cumulative dose of methylprednisolone acetate, there was a marked increase of liver enzymes, prompting immediate discontinuation of iv GC. Nevertheless, liver enzymes increased further, reaching a peak 45 days later, with values 30- to 50-fold greater than those prior to therapy, associated with evidence of impaired liver function. Liver biopsy showed a marked lymphocytic infiltration, likely indicating an autoimmune hepatitis. Based on the assumption that following GC-induced immune suppression, autoimmune hepatitis might have been precipitated by sudden re-activation of the immune system during interpulse periods, we treated the patient with im and then oral GC, in order to re-induce immune suppression. Within three days from re-institution of GC therapy, there was a marked reduction of liver enzymes and amelioration of liver function. Complete normalization was achieved two months later, while the patient was still receiving a low maintenance dose of oral prednisone.

The subdural evacuation port system: outcomes from a single institution experience and predictors of success.

PubMed

Neal, Matthew T; Hsu, Wesley; Urban, Jillian E; Angelo, Nicole M; Sweasey, Thomas A; Branch, Charles L

2013-06-01

Numerous surgical options for treatment of chronic subdural hematomas (cSDH) exist. Several reports have examined the Subdural Evacuating Port System (SEPS), a variation of the twist drill craniotomy (TDC) technique. Although high success rates have been reported, a significant portion of patients treated with SEPS fail and require additional procedures. This report examines the largest single institution experience with the SEPS and explores patient and imaging characteristics associated with successful procedures. A retrospective chart review was performed to identify all patients who have undergone SEPS drainage of cSDH. Demographic and radiographic characteristics were evaluated. Demographic data included patient's age, sex, presenting symptoms, pre-procedural GCS score, and use of anticoagulation or antiplatelet agents. The volume of drainage per procedure and radiographic data including laterality, density, and maximal diameter of the collection, presence of septations, midline shift, resolution of the collection 3 weeks post procedure, and measurements to assess atrophy were collected. Total length of stay and time in the intensive care unit was also recorded. Results were classified as a success or failure based on the need for additional procedures including craniotomy or burr hole craniotomy in the operating room. Patients treated with two SEPS procedures during the same hospitalization and no other procedures were included in the success group for statistical analyses. 171 subdural collections were treated in 159 patients (147 unilateral and 12 bilateral). One hundred thirty three collections (77.8%) were successfully drained. In a comparison of the success and failure groups, there were no statistically significant differences (p<0.05) in the patients' mean age, sex, presenting Glasgow Coma Scale score, coagulation profile, presenting symptoms (except altered mental status and language disturbance), subdural diameter or laterality, midline shift

Anti-thymocyte serum as part of an immunosuppressive regimen in treating haematological immune-mediated diseases in dogs.

PubMed

Cuq, B; Blois, S L; Mathews, K A

2017-06-01

To report the outcomes associated with the use of rabbit anti-dog thymocyte serum in dogs with haematological immune-mediated diseases. Medical records from 2000 to 2016 of patients diagnosed with immune-mediated haemolytic anaemia, immune-mediated thrombocytopenia, pancytopenia and myelofibrosis were reviewed. All dogs had a severe or refractory disease and received rabbit anti-dog thymocyte serum. Lymphocyte counts were used to monitor the immediate anti-thymocyte effect of therapy; long-term patient outcome was recorded. A total of 10 dogs were included. All dogs except one had a notable decrease in their lymphocyte count after rabbit anti-dog thymocyte serum; four of nine had a decrease to less than 10% of the initial lymphocyte count and one dog reached 10·8%. All dogs were discharged from the hospital following their treatment. The dog with no alteration of lymphocyte count following therapy with rabbit anti-dog thymocyte serum had refractory immune mediated haemolytic anemia and was euthanised within two weeks. All other cases achieved clinical remission with immunosuppressive therapy eventually being tapered (3 of 10) or discontinued (6 of 10). Rabbit anti-dog thymocyte serum therapy might be of interest as an adjunctive therapy in refractory immune-mediated diseases and suppressed lymphocyte counts in most dogs. © 2017 British Small Animal Veterinary Association.

A Case of Simultaneous Traumatic Dorsal Dislocation of All Five Metatarsophalangeal Joints Treated Successfully With Closed Reduction.

PubMed

Bhide, Pushkar P; Anantharaman, Chinnadurai; Mohan, Ganesan; Raju, Karuppanna

2016-01-01

Simultaneous dislocation of multiple metatarsophalangeal joints is a rare injury, because of the impediment presented by the anatomy of the lesser metatarsophalangeal joints. To the best of our knowledge, only 1 case of simultaneous dislocation of all 5 metatarsophalangeal joints has been previously reported in peer-reviewed studies. Owing to the same anatomic structures that obstruct relocation, closed reduction has been known to fail in a large proportion of cases. We report a case of simultaneous dorsal dislocation of all 5 metatarsophalangeal joints of the right foot after a motor vehicle accident. The highlight of our case was successful closed reduction after application of the reduction maneuver to all lesser metatarsophalangeal joints simultaneously in the second attempt with the patient under anesthesia. On confirming the stability of the reduction, the foot was immobilized in a short-leg, posterior slab cast for 3 weeks without placing Kirschner wires across the joints. At the 3-month follow-up evaluation, the patient had reacquired their preinjury level of activity with a good range of motion . At the 2-year follow-up evaluation, this range of motion was maintained with no radiologic evidence of arthrosis. We have inferred that the reduction was successful the second time because the maneuver freed the soft tissue structures from the contiguous impingement in the metatarsophalangeal joints by the exact reversal of the mode of injury using simultaneous application of the maneuver to all the lesser metatarsophalangeal joints. We encourage a trial of this modification of the closed reduction method in the emergency setting before proceeding to open reduction, because the results of closed reduction can be biologically rewarding without the risks associated with open surgical dissection. Copyright © 2016 American College of Foot and Ankle Surgeons. Published by Elsevier Inc. All rights reserved.

Fluidized bed heat treating system

DOE Office of Scientific and Technical Information (OSTI.GOV)

Ripley, Edward B; Pfennigwerth, Glenn L

Systems for heat treating materials are presented. The systems typically involve a fluidized bed that contains granulated heat treating material. In some embodiments a fluid, such as an inert gas, is flowed through the granulated heat treating medium, which homogenizes the temperature of the heat treating medium. In some embodiments the fluid may be heated in a heating vessel and flowed into the process chamber where the fluid is then flowed through the granulated heat treating medium. In some embodiments the heat treating material may be liquid or granulated heat treating material and the heat treating material may be circulatedmore » through a heating vessel into a process chamber where the heat treating material contacts the material to be heat treated. Microwave energy may be used to provide the source of heat for heat treating systems.« less

Nail psoriasis in an adult successfully treated with a series of herbal skin care products family – a case report.

PubMed

Tirant, M; Hercogovấ, J; Fioranelli, M; Gianfaldoni, S; Chokoeva, A A; Tchernev, G; Wollina, U; Novotny, F; Roccia, M G; Maximov, G K; França, K; Lotti, T

2016-01-01

Psoriasis is a common chronic inflammatory dermatosis that causes significant distress and morbidity. Approximately 50% of patients with cutaneous psoriasis and 90% of patients with psoriatic arthritis demonstrate nail involvement of their psoriasis. Left untreated, nail psoriasis may progress to debilitating nail disease that leads to not only impairment of function but also on quality of life. We report the case of a 50-year-old male patient with recalcitrant nail dystrophies on the fingers since the age of 40, who responded successfully to Dr. Michaels® product family. The patient had a 35-year history of plaque psoriasis localised on the scalp, ears, groin, limbs, and trunk and with psoriatic arthritis. The nail symptoms consisted of onycholysis, onychomycosis, leukonychia, transverse grooves, nail plate crumbling and paronychia of the periungal skin. This case represents the efficacy and safety of the Dr. Michaels® (Soratinex® and Nailinex®) product family with successful resolution of nail dystrophies and surrounding paronychia with no reported adverse events.