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1.
Kotani T Takeuchi T Makino S Hata K Yoshida S Nagai K Wakura D Shoda T Hanafusa T 《Clinical rheumatology》2011,30(8):1021-1028
We retrospectively examined the effect of combination therapy with prednisolone and cyclosporin-A (CSA) on the findings of
pulmonary function tests (PFTs) and chest high-resolution computed tomography (HRCTs) scans in patients with dermatomyositis
(DM) and acute/subacute interstitial pneumonia (A/SIP). We also examined whether CSA therapy improved PFT and chest HRCT findings.
DM patients (n = 14) with A/SIP were treated with 1 mg/kg/day prednisolone and 4 mg/kg/day CSA within 4.4 days (range, 1–12 days) from diagnosis.
The trough level (C0) and 2-h post-dose blood concentration (C2) of CSA were measured. PFTs and HRCT scans were performed
before and 1 year after treatment. The total ground-glass opacity area was calculated with the HRCT findings and used as the
CT score. Combination prednisolone and CSA therapy improved the TLC%, VC%, FVC%, EFV1.0%, and CT score (P = 0.027, 0.003, 0.002, 0.001, and 0.001, respectively). The C0 level was 178.8 ng/ml (range, 71–456 ng/ml), and the C2 level
was 1,336.6 ng/ml (range, 814–2,873 ng/ml). Therapeutic changes in FVC%, FEV1.0%, and DLCO% were correlated with the C2 CSA
level (P = 0.047, 0.025, and 0.035, respectively). However, the PFT results and CT scan scores did not correlate with the daily dose
or C0 level of CSA. Improvements in the CT score were correlated with time from IP diagnosis to CSA initiation (P = 0.014). Early intervention with prednisolone and CSA combination therapy and tight control of the daily CSA dose by monitoring
the C2 level improved PFT and chest HRCT findings in DM–A/SIP. 相似文献
2.
David Gómez-Almaguer José Carlos Jaime-Pérez Verónica Garza-Rodríguez Adrián Chapa-Rodríguez Luz Tarín-Arzaga José Luís Herrera-Garza Guillermo J. Ruiz-Argüelles Avril López-Otero Oscar González-Llano Laura Rodríguez-Romo 《Annals of hematology》2010,89(3):299-303
Aplastic anemia (AA) is most frequently due to autoimmune attack on its own stem cells. Alemtuzumab is a monoclonal antibody
which recognizes the CD52 antigen on the surface of T and B cells. It has proved useful in autoimmune diseases, lymphoproliferative
conditions, and graft versus host disease. Based on its immunosuppressive properties, we treated 14 AA patients with alemtuzumab.
Median age was 23 years. Ten milligrams of alemtuzumab were injected subcutaneously each day for five consecutive days. Cyclosporine
A was also administered orally at a dose of 2 mg/kg every 12 h for 3 months, and then gradually tapered. Response to alemtuzumab
was followed for a median of 20 months. There were eight responses (57.1%), two complete and six partial. Whereas six (42.8%)
patients were non-responders. Median complete blood count values on alemtuzumab responders were Hb 13.1 mg/dL, absolute neutrophil
count 2.4 × 109/L, and platelets 97.5 × 109/L. A good response was produced in 57% of AA patients with the administration of alemtuzumab, who lacked a stem cell donor. 相似文献
3.
The impact of tetrahydrobiopterin (BH4) treatment on phenylalanine tolerance, medical-food consumption, and nutrition status in patients with phenylketonuria (PKU)
was investigated. Six children (5–12 years) with well-controlled PKU, responding to a BH4 dose of 20 mg/kg per day, were assessed for 24 months. Mean dietary phenylalanine tolerance increased from 421 ± 128 to 1470 ± 455 mg/day.
Height Z scores significantly improved from 0.25 ± 0.99 at baseline to 0.53 ± 1.16 at 24 months (p for trend < 0.001). Patients
tolerated more phenylalanine and more intact protein and required less medical food (protein supplement). Improved linear
growth and nutrition status was seen over the course of the 24-month follow-up. Due to the variation in phenylalanine tolerance,
dietary recommendations should be tailored to the patient’s individual requirements. 相似文献
4.
Adiponectin levels correlate with the severity of hypertriglyceridaemia in glycogen storage disease Ia 总被引:1,自引:1,他引:0
R. H. J. Bandsma G. P. A. Smit D.-J. Reijngoud F. Kuipers 《Journal of inherited metabolic disease》2009,32(1):27-31
Summary
Background: The clinical severity of phenylalanine hydroxylase deficiency is usually defined by either pre-treatment phenylalanine (Phe)
concentration or Phe tolerance at 5 years of age. So far, little is known about the course of Phe tolerance or the ability
of both pre-treatment Phe and Phe tolerance at early age to predict Phe tolerance at later age.
Aim: This study was conducted to investigate the course of the individual Phe tolerance and to assess the predictive value of both
the pre-treatment Phe concentration and Phe tolerance at 1 and 6 months and 1, 2, 3 and 5 years for Phe tolerance at 10 years
of age.
Method: Data on blood Phe concentration, prescribed Phe intake and weight of 213 early and continuously treated Dutch PKU patients
up to 10 years of age were collected. Data acquired under good metabolic control were used in the study. Tolerance was expressed
in mg/day and mg/kg per day.
Results: Data at 1 and 6 months and at 1, 2, 3 and 5 years of 61, 58, 59, 57, 56 and 59 patients were included for comparison with
the Phe tolerance at 10 years. Phe tolerances (mg/kg per day) at 2, 3 and 5 years showed a clear correlation with the tolerance
at 10 years of age (r = 0.608, r = 0.725 and r = 0.661). Results for tolerance expressed as mg/day were comparable. Pre-treatment Phe concentrations did not correlate significantly
with the tolerance.
Conclusion: Pre-treatment Phe is unreliable but Phe tolerance is a reliable predictor of the tolerance at 10 years of age, starting at
2 years of age.
Competing interests: None declared
References to electronic databases: Phenylalanine hydroxylase: EC 1.14.16.1. 相似文献
5.
Ilowite N Porras O Reiff A Rudge S Punaro M Martin A Allen R Harville T Sun YN Bevirt T Aras G Appleton B 《Clinical rheumatology》2009,28(2):129-137
This study assessed the safety and preliminary efficacy of the interleukin-1 receptor antagonist anakinra in patients with
polyarticular-course juvenile rheumatoid arthritis (JRA). Eighty-six patients entered a 12-week open-label run-in phase (1 mg/kg
anakinra daily, ≤100 mg/day). Fifty responders were randomized to anakinra or placebo in a 16-week blinded phase, followed
by a 12-month open-label extension (N = 44). Due to low enrollment, the primary endpoint was changed from efficacy to safety. The incidence and nature of adverse
events were similar across all study phases, with the exception of injection site reactions, which were mild to moderate and
decreased with time. Anakinra produced a nonsignificant (P = 0.11) reduction in disease flares compared with placebo. When normalized to 1 mg/kg dose, anakinra plasma concentrations
were similar to values in adult patients with rheumatoid arthritis. These results indicate that anakinra 1 mg/kg once daily
(≤100 mg/day) is safe and well tolerated in patients with JRA. 相似文献
6.
Major TC Olszewski B Rosebury W Okerberg C Carlson T Ostroski R Schroeder R Kowala MC Leadley R 《Cardiovascular drugs and therapy / sponsored by the International Society of Cardiovascular Pharmacotherapy》2008,22(6):469-478
Introduction Controlling hypertension by angiotensin converting enzyme inhibitors (ACEI) or angiotensin receptor blockers (ARB), mechanisms
that inhibit later pathway steps in the renin–angiotensin system (RAS), have clinically afforded protection against cardiac
and renal disease.
Materials and methods In order to determine if blocking the RAS rate-limiting step of angiotensin II generation via renin inhibition could afford
similar end organ protection in a human-relevant preclinical model, this study investigated the cardiac and renal effects
of a nonpeptide, piperidine renin inhibitor (RI; 100 mg/kg/day PO) in double transgenic mice (dTGM) which express both human
renin and angiotensinogen genes. RI was compared to the ARB, candesartan (3 mg/kg/day PO), and to the ACEI, enalapril (60 mg/kg/day
PO) in a 4-week dosing paradigm. These doses of RI, ACEI and ARB were previously found to normalize mean blood pressure (MBP)
to 110 + 3, 109 + 7 and 107 + 6 mmHg, respectively, after 1 day of treatment.
Results and discussion In the dTGM, PRA, plasma aldosterone, GFR, microalbuminuria and left ventricular free wall thickness (LVH) were higher than
in the wild type C57BL/6 mice. Microalbuminuria and LVH were significantly reduced by 93% and 9% for the RI, 83% and 13% for
enalapril and 73% and 6% for candesartan, respectively. PRA and aldosterone were reduced by the RI 56% and 23%, respectively.
These results suggest that the RI provides protection against cardiac and renal disease, similar to ARB and ACEI. 相似文献
7.
Harvesting sufficient progenitor cells from bone marrow (BM) for pediatric patients is a challenging process, especially from
smaller donors. Growth factor administration to donors prior to harvest results in an enrichment of the graft and leads to
early engraftment. A total of 41 patients received a human leukocyte antigen-identical sibling transplantation using granulocyte
colony-stimulating factor (G-CSF)-primed BM. All donors received G-CSF 10 μg/kg/day for 2 days prior to harvest. The median
weight difference between donor and recipient was 3.9 kg (range, −29.8 to 32 kg), and the median number of CD34+ cells harvested was 4.16 × 106/kg (range, 1.17–31.9 × 106/kg). The median time to neutrophil engraftment was 12 days (range, 10–27 days), and the time for platelet engraftment was
20 days (range, 12–64 days). The cumulative incidence of acute grade 2 to 3 graft-versus-host disease (GVHD) and chronic GVHD
was 4.9% and 5.1%, respectively. An analysis according to the weight difference between donor and recipient showed there was
no significant difference in harvested CD34+ cell dose and in time required for engraftment between smaller and heavier donor recipients. G-CSF-primed BM allows successful
engraftment and provides a valuable alternative to unstimulated BM and peripheral blood stem cells with good engraftment and
tolerable GVHD even in patients with smaller donors. 相似文献
8.
Plasmodium falciparum infection transmitted by transfusion: A cause of hemophagocytic syndrome after bone marrow tranplantation in a non‐endemic country
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Saloua Ladeb Nour Ben Abdejlil Najla Fakhfakh Amel Lakhal Dorra Belloumi Leila Ben Hamed Aicha Kallel Lamia Torjman Rym El Fatimi Slama Hmida Kalthoum Kallel Tarek Ben Othman 《Transplant infectious disease》2018,20(3)
A 27‐year‐old man with severe aplastic anemia underwent bone marrow transplantation from his HLA identical brother in July 2016. Conditioning included ATGAM 30 mg/kg for 3 days and Cyclophosphamide 50 mg/kg for 4 days. The patient received several platelet and red blood cell transfusions before and after the conditioning. The patient received broad spectrum antibiotics and caspofungin because persistant febrile neutropenia without bacteriological or mycological documentation. Hemophagocytic syndrome was diagnosed on day +12. Steroids at 1 mg/kg were started on day +12. Fever resolved the same day but resumed 3 days later associated to intravascular hemolysis with no schizocytes on blood smears and negative DAT. Thick blood film smears performed on day +26 revealed Plasmodium falciparum parasites (parasitemia = 20%). Except the level of parasitemia, there were no signs of gravity. Quinine was started on day 26 at a loading dose of 15 mg/kg followed by 8 mg/kg three times a day for 20 doses. Fever vanished after 2 days. Parasitemia cleared in 3 days and remained negative thereafter. Investigations revealed that the patient was transfused by a red cell unit harvested in a voluntary donor native of a malaria endemic country. PCR for P. falciparum performed in this donor in the frame of investigations was positive. The patient is alive with a normal blood count 1 year after BMT. 相似文献
9.
Baijun Fang Ning Li Yongping Song Qin Han Robert Chunhua Zhao 《Annals of hematology》2010,89(11):1099-1105
We investigated the efficacy of the induction therapy involving granulocyte colony-stimulating factor (G-CSF) and low-dose
homoharringtonine as well as standard-dose imatinib, which we called the G-CSF + homoharringtonine + imatinib (GHI) regimen,
in patients with chronic myelogenous leukemia (CML) in blast crisis who have failed prior single-agent therapy with imatinib.
Twelve patients were enrolled. The GHI regimen consisted in a unique induction course where imatinib was administered at 400 mg day−1 until remission, together with homoharringtonine (1 mg/m2 s.c. twice daily for 14 days every 28 days), and G-CSF, which was administered 1 day before chemotherapy (5 μg/kg s.c. daily).
Patients who failed to obtain at least a partial hematologic response (PHR) after three courses were taken off study. Patients
who responded to induction treatment and who had a matched donor received allogeneic hematopoietic stem cell transplantation
(allo-HSCT). The results demonstrates that the GHI regimen re-induce hematologic responses or improve the cytogenetic responses
in all evaluable patients. Furthermore, eligible patients have benefited from allo-HSCT after response to this induction treatment.
We conclude that the GHI regimen may overcome disease-poor response to conventional doses of imatinib and this approach deserves
further evaluation as frontline therapy for newly diagnosed CML. 相似文献
10.
T. Danne H. W. de Valk T. Kracht K. Walte R. Geldmacher L. Sölter W. von dem Berge Z. K. Welsh J. R. Bugler K. Lange O. Kordonouri 《Diabetologia》2009,52(8):1496-1503
Aims/hypothesis This study was designed to investigate the use and impact of a continuous glucose monitoring system (the FreeStyle Navigator)
under home-use conditions in the self-management of type 1 diabetes.
Methods A 20 day masked phase, when real-time data and alarms were not available, was compared with a subsequent 40 day unmasked phase
for a number of specified measures of glycaemic variability. HbA1c (measured by DCA 2000) and a hypoglycaemia fear survey were recorded at the start and end of the study.
Results The study included 48 patients with type 1 diabetes (mean age 35.7 ± 10.9, range 18–61 years; diabetes duration 17.0 ± 9.5 years).
Two patients did not complete the study for personal reasons. Comparing masked (all 20 days) and unmasked (last 20 days) phases,
the following reductions were seen: time outside euglycaemia from 11.0 to 9.5 h/day (p = 0.002); glucose SD from 3.5 to 3.2 mmol/l (p < 0.001); hyperglycaemic time (>10.0 mmol/l) from 10.3 to 8.9 h/day (p = 0.0035); mean amplitude of glycaemic excursions (peak to nadir) down by 10% (p < 0.001); high blood glucose index down by 18% (p = 0.0014); and glycaemic risk assessment diabetes equation score down by 12% (p = 0.0013). Hypoglycaemic time (<3.9 mmol/l) decreased from 0.70 to 0.64 h/day without statistical significance (p > 0.05). Mean HbA1c fell from 7.6 ± 1.1% at baseline to 7.1 ± 1.1% (p < 0.001). In the hypoglycaemia fear survey, the patients tended to take less snacks at night-time after wearing the sensor.
Conclusions/interpretation Home use of a continuous glucose monitoring system has a positive effect on the self-management of diabetes. Thus, continuous
glucose monitoring may be a useful tool to decrease glycaemic variability. 相似文献
11.
Summary A patient with recurrent episodes of hyperammonaemia (highest ammonia level recorded 229 μmol/L, normal 9–33) leading to
altered levels of consciousness was diagnosed with partial N-acetylglutamate synthase (NAGS) deficiency (9% residual activity) at age 5 years and was treated with ammonia-conjugating
agents (Ucephan 250 mg/kg per day and later sodium phenylbutyrate 200–250 mg/kg per day) for 15 years. A chronically low
serum carnitine level (pretreatment plasma free carnitine 4 nmol/L, normal 37 ± 8 nmol/L; total carnitine 8 nmol/L, normal
46 ± 10) was assumed to be secondary and was treated with supplemental carnitine (30–50 mg/kg per day). Hypoglycaemia (blood
sugar 35 mg/dl, normal 70–100), cardiomegaly, and fatty liver were also noted at diagnosis. The patient died unexpectedly
at age 20 years. In retrospect, it was learned that the patient had stopped his carnitine without medical consultation several
weeks prior to his death. Additional molecular investigations identified two mutations (R254X and IVS3 + 1G > A) in the patient’s
OCTN2 (SLC22A5) gene, consistent with a diagnosis of primary carnitine deficiency due to carnitine transporter defect. R245X is a founder
mutation in Southern Chinese populations. It is unknown whether the original NAGS deficiency was primary or secondary, but
molecular analysis of the NAGS gene failed to identify mutations. Urea cycle enzyme expression may be affected by fatty acid
suppression of an AP-1 binding site in the promoter enhancer region of the urea cycle gene. Regardless, it is clear that the
NAGS abnormality has led to delay of recognition of the OCTN2 defect, and modified the clinical course in this patient.
Electronic Supplementary Material The online version of this article (doi:) contains supplementary material, which is available to authorized users.
Online citation: JIMD Short Report #074 (2007) Online 相似文献
12.
N. Kröger W. Zeller H. T. Hassan W. Krüger H. Renges K. Hummel K. Gutensohn C. Lölliger A. R. Zander 《Annals of hematology》1998,76(6):257-262
We investigated the feasibility of mobilizing peripheral blood stem cells (PBSC) with G-CSF alone in 24 patients with multiple
myeloma. The median age was 53 years (range 33–62). All patients had stage II/III disease and responded to standard first-line
(n=6) or salvage chemotherapy (n=18). The median number of previous chemotherapy cycles was 7 (4–18) and the median number of prior melphalan-cycles was 6
(0–14). Nine (35%) patients had experienced prior radiation therapy. The patients received either 10 μg/kg G-CSF (n=18) or 24 μg/kg G-CSF (n=7, including one patient with previous 10 μg/kg G-CSF stimulation) daily s.c. for 5 or more consecutive days until completion
of harvesting, starting apheresis on the fifth day. G-CSF treatment was well tolerated, with only slight bone pain in half
of the patients (51%). After a median of three (range 1–7) apheresis procedures, medians of 3.8 (0.3–17)×106 CD34+ cells/kg, 8.5 (4.5–24)×108 MNC/kg, 2.9 (0.6–39.4)×104 CFU-GM/kg, and 5.6 (0.9–49)×104 BFU-E/kg were harvested. Three patients (12%) with extensive melphalan pretreatment failed the target collection of at least
2.0×106 CD34+ cell/kg. Pretreatment with six or more cycles of melphalan yielded a smaller number of CD34+ cells than pretreatment
with fewer than six cycles (2.5 vs 5.3×106/kg;p=0.001). Nineteen patients underwent high-dose chemotherapy consisting of either total marrow irradiation (9 Gy)/busulfan
(12 mg/kg) and cyclophosphamide (120 mg/kg) (n=10), or busulfan (14 mg/kg)/cyclophosphamide (120 mg/kg) (n=5), or tandem melphalan (200 mg/m2). The median time for granulocyte (>1.0/nl) and platelet (>50/nl) recovery was 10 and 14 days (ranges 7–12 and 8–40), respectively.
G-CSF alone is a safe, alternative approach to mobilizing sufficient PBSC in patients with multiple myeloma and allows an
exact prediction of harvest time. G-CSF-mobilized PBSCs ensure rapid engraftment after myeloablative therapy. Melphalan treatment
should be avoided in patients who are candidates for high-dose chemotherapy.
Received: February 5, 1998 / Accepted: April 14, 1998 相似文献
13.
H. C. Schouten J. J. M. Raemaekers J. C. Kluin-Nelemans H. van Kamp W. A. M. Mellink M. B. van't Veer 《Annals of hematology》1996,73(6):273-277
Purpose: To analyze whether, in addition to survival, and disease-free survival progression-free interval after transplantation would
be longer than the last progression-free interval before transplantation, supporting the argument that high-dose therapy may
change the biologic behavior of the disease.
Patients and methods: Patients with a poor-risk relapsed follicular NHL were treated with three cycles of doxorubicin 50 mg/m2 and teniposide
60 mg/m2, followed by etoposide 350 mg/m2, cyclophosphamide 60 mg/kg, and TBI and unpurged BMT.
Results: Twelve patients were entered in the study. Ten patients fulfilled the criteria for response and underwent transplantation,
two of them with an allograft. Nine of ten patients with transplants achieved a complete remission after BMT. One patient
died on day 41 due to veno-occlusive disease. The nine patients with transplants who were evaluable for follow-up had a conversion
of remission or response duration after transplantation, their progression-free interval after BMT being superior to the last
one before BMT with a median of 1044+ days. Overall survival and disease-free survival in the transplant patients after a
median follow-up of 1160 days from BMT is 90%.
Conclusion: High dose chemotherapy followed by stem cell rescue may change the clinical course in follicular non-Hodgkin's lymphoma
patients.
Received: 20 August 1996 / Accepted: 25 September 1996 相似文献
14.
Maeshima E Kida Y Goda M Minami Y 《Modern rheumatology / the Japan Rheumatism Association》2006,16(4):239-242
We describe a case where intermittent and continuous administrations of a small amount of immune globulin were effective in
the treatment of refractory chronic immune thrombocytopenic purpura by systemic lupus erythematosus (SLE). Steroid pulse therapy
and cyclophosphamide pulse therapy were considered for thrombopenia. However, the patient had compressed fracture of the lumbar
vertebrae due to osteoporosis and right external malleolus ulcer with complications of infection. Therefore, high-dose intravenous
immune globulin (IVIG) therapy (400 mg/kg daily for 5 consecutive days) was administered. Then, as a maintenance therapy,
a small amount of 400 mg/kg for 1 day (400 mg/kg monthly) was given in an intermittent and continuous manner, which resulted
in improvement of thrombocytopenia and reduction of the amount of steroid administered. 相似文献
15.
D. Papamichael T. Andrews D. Owen M. Carter J. Amess T. A. Lister A. Z. Rohatiner 《Annals of hematology》1996,73(6):259-263
Twenty-six patients with newly diagnosed ALL (age range 15–49 years, median 32 years) received treatment comprising: cycles
1 and 2: adriamycin 30 mg/m2 days 1–3, vincristine: 2 mg days 1, 8, and 15, with prednisolone 40 mg daily, given until complete remission (CR). l-asparaginase 10000 units/m2, days 1–14, was given only with the first cycle. Cycle 3 consisted of 100 mg/m2 etoposide orally, days 1–5, and 1 gm/m2 bd cytosine arabinoside (ara-C) days 1–5. Cycles 1–3 were then repeated. Intrathecal methotrexate (MTX) 12.5 mg was given
on day 1 of each treatment cycle. The first 12 consecutive patients received this chemotherapy alone, the subsequent 14 received,
in addition, 3 μg/kg GM-CSF subcutaneously, from day 4 of cycles 1, 2, 4, and 5 (and from day 6 of cycles 3 and 6) until the
absolute neutrophil count had reached 0.5×109/l. All patients in whom CR was achieved then received prophylactic cranial irradiation. With the exception of those with
T-ALL, this was followed by oral maintenance therapy consisting of 6-mercaptopurine, MTX, and cyclophosphamide for 3 years.
Patients receiving GM-CSF did not have shorter intercycle times or a lower incidence of documented infections than those who
did not receive it. The CR rate was 89% overall - uninfluenced by GM-CSF, but higher than that achieved previously at St Bartholomew's
Hospital in an equivalent age-group.
Received: 15 April 1996 / Accepted: 2 September 1996 相似文献
16.
Inusha Panigrahi Ram K. Marwaha Rashmi R. Das Amita Trehan Deepak Bansal 《Annals of hematology》2010,89(2):231-140
Deferiprone (L1) has been used in several countries for iron chelation therapy for over one decade. Long-term results on the
drug are lacking. In the present study, data of 110 patients on deferiprone (L1) for up to 17 years were analyzed. On a mean
L1 dose of 70.2 mg/kg/day (range 44–100), serum ferritin level showed a very steady decrease with time from an initial mean
(±SD) of 3,033.61 ± 1,468.04 ng/ml to final of 1,665.08 ± 949.93 ng/ml after a mean (±SD) of 6.1 ± 3.8 years. In total, 13
patients discontinued L1 therapy. Major complications of L1 requiring permanent discontinuation of treatment included arthropathy
(n = 8, 7.2%) and neutropenia/agranulocytosis (n = 5, 4.5%). Lesser complications permitting continued L1 treatment included transient mild leucopenia or thrombocytopenia
(n = 3) and gastrointestinal problems (n = 5). There were a total of three deaths attributed to agranulocytosis. Although the complications associated with L1 treatment
are significant and require close monitoring, they do not preclude effective long-term therapy in the vast majority of patients.
A longer duration of therapy is required for effective response in chronically iron-overloaded patients. Further well-controlled
prospective studies of L1 are required to identify factors affecting individual response to therapy. 相似文献
17.
To evaluate the effect of intra-articular steroid injection (IASI) of betamethasone acetate/betamethasone sodium phosphate
(Celestone Chronodose) at the knee joint on blood glucose and fructosamine levels in controlled diabetic patients with osteoarthritis
of the knee. Controlled diabetic patients (HgA1C < 7) with symptomatic osteoarthritis of the knee who failed medical and physical
therapy and use modern versions of home-monitoring blood glucose devices were offered an IASI of Celestone Chronodose at the
knee joint. If agreed, patients were asked to document blood glucose levels before and 2 h after meals for 1 week prior to
IASI and daily for 4 days then every other day for 10 days following IASI of 1 ml of Celestone Chronodose. Serum fructosamine
levels were obtained just prior and 2 weeks following the injection. Six patients completed the study and all had a significant
brisk elevation of blood glucose levels within the first hour lasting for less than 48 h in five of them. Mean glucose level
1 h following the injection was 218.33 ± 41.75 mg% and mean maximal level 322.5 ± 67.75 mg%. There was no significant change
in fructosamine levels. IASI of Celestone Chronodose will transiently increase blood glucose levels in diabetic patients.
It has no significant effect on serum fructosamine levels. 相似文献
18.
An unusual case of systemic lupus erythematosus, lupus nephritis, and transient monoclonal gammopathy 总被引:1,自引:0,他引:1
A 23-year-old female patient suffering from active systemic lupus erythematosus (SLE) was treated with azathioprine (2 mg/kg
per day) and prednisone. Lupus nephritis class III with increasing proteinuria developed 28 months after disease onset. Treatment
was switched to monthly pulse cyclophosphamide administered intravenously for 6 months (total dose 6.3 g), followed by oral
azathioprine and low-dose prednisone to maintain partial remission. Eight months later, the patient developed an acute exacerbation
of SLE with fever, proteinuria of 9.1 g/day, pancytopenia, and cerebral involvement with cephalgias and a grand mal seizure.
She responded well to high-dose corticosteroids (500 mg prednisolone pulses over 3 days, i.v.) and was switched from azathioprine
to methotrexate (12.5–15 mg per week). Under this treatment, lupus activity gradually decreased and the patient felt well
again. Five years after the initial diagnosis of SLE, a rapidly increasing immunoglobulin G-kappa type (IgG-κ) monoclonal
gammopathy developed, reaching a maximal serum paraprotein concentration of 73.5 g/l. Bone marrow biopsy revealed 15% of moderately
abnormal, highly differentiated plasma cells arranged in small clusters and expressing IgG-κ. No bony lesions were detectable
on skeletal radiographs. Pulses of dexamethasone (40 mg) were administered and led to a transient decrease of paraproteinemia
to a minimum of 31.9 g/l, followed by an increase to 62 g/l. At that point, high-dose chemotherapy supported by autologous
stem cell transplantation was considered. Due to an intermittent pneumococcal septicemia, methotrexate was discontinued and
dexamethasone was replaced by 5–10 mg cloprednol. At this point, totally unexpectedly, the paraprotein decreased spontaneously
without any further cytostatic treatment and was no longer detectable 1 year later. Concomitantly, plasma cell counts in bone
marrow biopsies fell to below 5%. As SLE remained inactive, the patient became pregnant and gave birth to a healthy child.
During late pregnancy, SLE activity flared up with rising proteinuria and blood pressure. Therefore, after delivery, cyclophosphamide
(100 mg/day, orally) was readministered for 4 months, resulting in an improvement of kidney function with stable proteinuria
of 1–2 g/l to date. Paraproteins are no longer detectable. In conclusion, this case report documents the rare event of transient
paraproteinemia in a patient with SLE. A self-limiting regulatory defect in the control of a terminally differentiated B-cell
clone may be the origin of this phenomenon.
Received: 14 April 2000 / Accepted: 16 June 2000 相似文献
19.
The changes in serum chemokines following leflunomide therapy in patients with rheumatoid arthritis 总被引:1,自引:0,他引:1
We undertook this study to evaluate the effects of leflunomide, an oral pyrimidine synthesis inhibitor, on the serum chemokine
levels in patients with active rheumatoid arthritis (RA) who were refractory to treatment with methotrexate (MTX) or did not
tolerated MTX treatment. RA patients were supposed to receive leflunomide (100 mg/day loading dose for 3 days followed by
20 mg/day orally for the 12 months). Serum concentrations of RANTES (regulated upon activation, normal T cell expressed and
secreted), monocyte chemoattractant protein-1 (MCP-1), and interleukin-8 (IL-8) were assessed by enzyme-linked immunosorbent
assay before and after 3, 6, 9, and 12 months of treatment with leflunomide. Three months therapy with leflunomide caused
reduction in serum RANTES and MCP-1 (in both cases, p < 0.001) levels. Decrease in the concentration of these chemokines persisted until the end of the study period but was less
significant. In the case of IL-8, its serum levels significantly diminished after 6 months of therapy with leflunomide (p < 0.01) and remained stable to the end of the study. Changes in serum chemokine levels were accompanied by significant decrease
of disease activity score (DAS; p < 0.001). Prior to the first dose of leflunomide, serum concentrations of studied chemokines correlated with marker of RA
activity such as the erythrocyte sedimentation rate and IL-8 level with DAS. Furthermore, we demonstrated significant correlations
between serum levels of RANTES, MCP-1, and IL-8. During study period, such associations were far less or not significant.
Leflunomide, beside a clinical improvement, reduce serum chemokines concentrations in RA patients. Leflunomide seems to be
an effective treatment for RA, alternative to current therapies. 相似文献
20.
Jytte Bieber Nielsen Karin E. Nielsen Flemming Güttler 《Journal of inherited metabolic disease》2010,33(1):9-16
Phenylketonuria (PKU) is an inherited metabolic disease characterized by phenylalanine (Phe) accumulation due to defects in
the enzyme phenylalanine hydroxylase (PAH). Phe accumulation can lead to cognitive impairment. Some individuals with PKU respond
to tetrahydrobiopterin (BH4) treatment, the natural cofactor of PAH, by a reduction in blood Phe concentrations. We tested
12 patients with PKU, 8–29 years of age, all carrying the common Y414C mutation in the PAH gene. Three were homozygous and nine were compound heterozygous, with the second mutation being a putative null mutation.
During the study period, genuine protein was increased to approximately 1 g/kg. The patients were treated with 20, 10, and
5 mg BH4/kg/day for 1 week on each dose, starting with 20 mg/kg. A positive response was defined as a decline in blood Phe > 30%.
Blood Phe was measured four times a week. Nonresponding children were excluded from the study. Eleven of 12 patients had a
positive response with 20 mg/kg, 5/10 responded on 10 mg/kg, and 1/9 on 5 mg/kg. Two were late responders, with a response
on 20 mg/kg after >48 h. We could confirm the previously reported inconsistent responsiveness of Y414C in the nine heterozygous
patients, whereas the three homozygous patients had early median Phe declines of 73%, 51%, and 27%, respectively, on the three
different doses. The varying responses despite uniform trial conditions and genotypes may be due to individual differences
in BH4 absorption or metabolism. No side effects were observed. 相似文献