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Pulse Therapy (pulse + therapy)

Distribution by Scientific Domains

Medical Sciences	100%

Distribution within Medical Sciences

Dermatology	34%
Transplantation	12%
Pathology	6%

Kinds of Pulse Therapy

methylprednisolone pulse therapy

steroid pulse therapy

Selected Abstracts

Dexamethasone pulse therapy in pemphigus

JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 6 2002
Gg Tóth
ABSTRACT Pulse therapy with high-dose glucocorticoids was introduced 20 years ago as a treatment modality for autoimmune disease and transplant rejection. The most popular dermatological indication for pulse therapy is severe pemphigus. We reviewed the sequelae of 14 patients with pemphigus who were treated by pulse therapy. Seven of them reached complete remission, although three of them needed a new pulse course due to disease flare-up. Adverse events were minor and confined to 60% of all patients: temporary facial flushing during pulse administration, sleep disturbances during the first night after pulse administration, and mood changes occurred during the week of pulse therapy. The study showed the possibility of oral instead of an intravenous route of dexamethasone pulse administration, which makes double-blind placebo-controlled trials ethically feasible. Fifty per cent of the patients reached complete remission. This retrospective study does not allow claims on the steroid-sparing effect. [source]

Extranodal NK/T-cell lymphoma, nasal type, presenting after 5 years of remission

INTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 3 2008
Tomonobu Ito MD
A 76-year-old woman with multiple edematous erythemas, erosions, and ulcers on the breast and abdomen was admitted to our hospital in June 2005. She had developed granulomatous bleeding lesions in the right nostril 6 years prior to her visit to our dermatology unit. She had been observed at the otorhinolaryngology department of our hospital, and a biopsy was taken from the nasal lesion. Computerized tomography and gallium scintigraphy (67Ga single-photon emission computed tomography) did not reveal any lesions corresponding to the diagnosis of malignant lymphoma. The histologic examination of the nasal specimen rendered a diagnosis of natural killer (NK)/T-cell lymphoma, nasal. Because imaging analysis indicated a small-sized tumor without metastases, oral prednisolone at 20 mg/day was administered for 1 month. The tumor decreased in size and disappeared after 19 months of low-dose steroid therapy. ,Five years after the initial treatment, the patient developed a fever of 38 °C with infiltrated erythemas and erosions on her breast. Erysipelas was initially suspected, but the antimicrobial agent did not show any effect and the multiple infiltrated erythemas and ulcers spread throughout her chest and abdomen (Fig. 1). The lymph nodes were not palpable. The right nasal cavity showed no granulomatous lesions or other signs of abnormality. The peripheral white blood cell count (3000/µL), red blood cell count (3.54 × 106/µL), and platelet count (112 × 103/µL) were reduced. Atypical lymphocytes were not observed. The serum lactic dehydrogenase (LDH; 1770 U/L; normal, 224,454 U/L), aspartate aminotransferase (AST; 140 U/L; normal, 10,30 U/L), and alanine aminotransferase (ALT; 57 U/L; normal, 3,29 U/L) levels were elevated. The soluble interleukin-2 (IL-2) receptor level was high (25,300 U/mL; normal, 167,497 U/mL). Epstein,Barr virus (EBV) serologic examination showed the immunoglobulin G (IgG) viral capsid antigen (VCA) at 1 : 320 and the EBV nuclear antigen (EBNA) at 1 : 40. IgM VCA and EBV early antigen-diffuse restricted antibody (EA) IgA and IgG were not detectable. Histologic findings from the left chest skin showed a distribution of atypical lymphocytes from the upper dermis to the subcutaneous tissue, and many foamy cells which had phagocytosed the hemocytes (Fig. 2a,b). Immunohistochemical analysis showed that the atypical lymphocytes were sCD3,, CD4,, CD8,, CD20,, CD56+, granzyme B+, and T-cell intracellular antigen (TIA-1) positive. Furthermore, EBV-encoded small RNAs (EBER), detected by in situ hybridization, exhibited a strong signal. The nasal lesions biopsied 6 years previously showed an identical staining pattern with the skin lesions immunohistochemically. Analysis of the T-cell receptor-, (TCR-,), TCR-,, and TCR-, gene did not reveal any clonal rearrangements, but the EBV gene was detected from the skin specimens by Southern blotting. Our patient's condition was diagnosed as a case of extranodal NK/T-cell lymphoma, nasal type, but the patient had concomitantly developed hemophagocytic syndrome (HPS). She was treated with a combination of steroid pulse therapy and chemotherapy (pirarubicin hydrochloride 30 mg/m2, cyclophosphamide 500 mg/m2, vincristine 1 mg/m2, prednisolone 30 mg/m2, etoposide 80 mg/m2). After the first session of chemotherapy, the lesions on the chest and abdomen diminished, but, 2 weeks later, the skin lesions recurred, and disseminated intravascular coagulation (DIC) induced by HPS supervened. The patient died as a result of multiple organ failure induced by HPS. Figure 1. Multiple infiltrated erythemas, erosions, and ulcers on the breast and abdomen Figure 2. Histologic findings of a skin biopsy specimen from the left chest (hematoxylin and eosin staining). (a) Dense infiltration of atypical lymphocytes from the upper dermis to the subcutaneous tissue (×40). (b) Many foamy cells had phagocytosed the hemocytes (×400) [source]

Intravenous dexamethasone-cyclophosphamide pulse therapy in comparison with oral methylprednisolone-azathioprine therapy in patients with pemphigus: Results of a multicenter prospectively randomized study

JOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT, Issue 3 2005
Intravenöse Dexamethason-Cyclophosphamid-Pulstherapie im Vergleich zu einer oralen Methylprednisolon-Azathioprin-Therapie bei Patienten mit Pemphigus-Erkrankungen: Ergebnisse einer multizentrischen, prospektiven, randomisierten Studie
Azathioprin; Cyclophosphamid; Pemphigus; Pulstherapie Summary Background: Pemphigus is a potentially life-threatening autoimmune blistering skin disease usually treated with high-dose corticosteroids in combination with immunosuppressive drugs. In a multicenter, prospectively randomized study we compared efficacy and side effects of a dexamethasone-cyclophosphamide (D/C) pulse therapy with a methylprednisolone-azathioprine (M/A) therapy in 22,patients with newly diagnosed pemphigus vulgaris and pemphigus foliaceus. Patients and methods: The 11,patients of the M/A group were treated with daily doses of methylprednisolone (initially 2,mg/kg body weight) and azathioprine (2,,,2,5,mg/kg body weight) which were subsequently tapered. D/C pulse therapy in 11,patients consisted of intravenous administration of 100,mg dexamethasone/d on 3 consecutive days along with cyclophosphamide (500,mg) on day one. Pulses were initially repeated every 2,,,4 weeks and then at increasing intervals. In between the pulses, oral cyclophosphamide (50,mg) was given daily for 6,months. Results: Within 24,months after treatment initiation, 5/11,patients of the D/C group had a remission (complete remissions after discontinuation of therapy in 3,patients) and 6/11,patients had a progression. In the M/A group, there were remissions in 9/11,patients (complete remissions after discontinuation of therapy in 3,patients) and progression in 1/11,patients. There were more relapses in M/A therapy after remission than in D/C therapy. Side effects were more common in the M/A group. These differences were not significant (p > 0,05). Conclusion: Because of the high number of progressions in patients treated with D/C therapy, we can not confirm the encouraging results of earlier reports about pulse D/C therapy. Nevertheless D/C therapy seemed to be better tolerated and, in case of primary efficacy, was associated with fewer recurrences than M/A therapy. Zusammenfassung Hintergrund: Pemphiguserkrankungen sind potentiell lebensbedrohliche blasenbildende Autoimmunerkrankungen, die üblicherweise mit hochdosierten Kortikosteroiden in Kombination mit Immunsuppressiva behandelt werden. In einer multizentrischen, prospektiven, randomisierten Studie verglichen wir die Wirksamkeit und Nebenwirkungen einer Dexamethason-Cyclophosphamid (D/C)-Pulstherapie mit einer Methylprednisolon-Azathioprin (M/A)-Therapie bei 22,Patienten mit neu diagnostiziertem Pemphigus vulgaris und Pemphigus foliaceus. Patienten und Methoden: 11,Patienten der M/A-Gruppe wurden kontinuierlich oral mit Methylprednisolon (initial 2,mg/kg Körpergewicht/Tag) und Azathioprin (2,,,2,5,mg/kg Körpergewicht/Tag) behandelt; die Dosen wurden schrittweise reduziert. Die Therapie bei den 11,Patienten der D/C-Gruppe erfolgte durch intravenöse Gabe von 100,mg Dexamethason/Tag an 3 aufeinander folgenden Tagen und 500,mg Cyclophosphamid am ersten Tag. Die Pulstherapie wurde zunächst alle 2,,,4 Wochen, dann in längeren Abständen wiederholt. Im Intervall wurden 50,mg Cyclophosphamid/Tag oral für 6,Monate verabreicht. Ergebnisse: Innerhalb von 24,Monaten nach Therapiebeginn kam es bei 5 von 11,Patienten der D/C-Gruppe zu einer Remission (komplette Remission nach Absetzen der Therapie bei 3,Patienten); bei 6 der 11,Patienten verlief die Erkrankung progredient. In der M/A-Gruppe kam es bei 9 von 11,Patienten zu einer Remission (komplette Remission nach Absetzen der Therapie bei 3,Patienten) und bei einem Patienten zu einer Progression. In der M/A-Gruppe traten häufiger Rezidive nach Remission auf als in der D/C-Gruppe. Therapienebenwirkungen kamen in der M/A-Gruppe häufiger vor. Diese Unterschiede waren nicht signifikant (p > 0,05). Schlußfolgerungen: Aufgrund der hohen Anzahl von Progressionen bei Patienten der D/C-Gruppe können wir die positiven Ergebnisse früherer Berichte über die D/C-Pulstherapie nicht bestätigen. Dennoch scheint die D/C-Therapie, beim einzelnen Patienten einmal erfolgreich, seltener zu Rezidiven zu führen und möglicherweise auch besser verträglich zu sein als die M/A-Therapie. [source]

Oral pemphigus: long term behaviour and clinical response to treatment with deflazacort in sixteen cases

JOURNAL OF ORAL PATHOLOGY & MEDICINE, Issue 4 2000
Michele D. Mignogna
Abstract: Systemic corticosteroids remain the mainstay of therapy for pemphigus. Their use has transformed what was almost invariably a fatal illness into one whose mortality is now below 10%. Unfortunately, the high doses and prolonged administration of corticosteroids that are often needed to control the disease result in numerous side effects, many of which are serious or even life-threatening. Sixteen patients affected by oral pemphigus vulgaris were retrospectively examined to illustrate the natural course of the disease and to describe the efficacy of the treatment we utilised. Deflazacort, used with azathioprine, is the steroid of first choice in our therapeutic protocols, while cyclophosphamide and methylprednisolone "pulse therapy" are reserved for cases unresponsive to high doses of oral corticosteroids. In addition, the literature on oral pemphigus vulgaris was reviewed with respect to clinical history, signs and symptoms, management, and treatment outcome. [source]

Toenail abnormalities and onychomycosis in chronic venous insufficiency of the legs: should we treat?

JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 3 2008
A Shemer
Abstract Background Toenail manifestations of chronic venous insufficiency (CVI) may often mimic the nail changes of onychomycosis. The current study aims to determine the frequency of toenails deformations in patients with CVI, onychomycosis prevalence among deformed toenails and the outcome of itraconazole treatment. Methods Patients with clinical evidence of (CVI) were enrolled. All patients with toenails deformations and proven onychomycosis were treated by itraconazole pulse therapy for 4 months and then followed-up for additional 5 to 6 months. Results Forty-four patients with CVI entered the study. Thirty-seven patients (84%) had nail deformations; 28 of 37 patients (75%) had onychomycosis; and 24 patients completed the follow-up period. Total cure was achieved in 6 of 24 patients (25%), and 14 patients (58%) had no improvement. Patients' age and CVI duration were significantly correlated to onychomycosis cure rate. Conclusions Nail deformations are more prevalent in CVI patients (84%) then in the general elderly population, and 75% of affected nails had also onychomycosis. In these patients, itraconazole achieved only 25% total cure rate compared with the 60% to 70% cure rate commonly cited in the literature, probably due to irreversible nail deformity caused by the CVI and due to the thickened nail that prevented penetrance of itraconazole into the nail plate. Therefore, before antifungal treatment is started for onychomycosis in patients with CVI, especially in older patients, the caregiver must stress out that the final outcome might not be as desirable as in pure onychomycosis patients. [source]

Pulse itraconazole vs. continuous terbinafine for the treatment of dermatophyte toenail onychomycosis in patients with diabetes mellitus

JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 10 2006
AK Gupta
Abstract Background, Oral terbinafine and oral itraconazole are two of the most common agents used for the treatment of toenail dermatophyte onychomycosis. Despite the fact that diabetic patients are more likely to have onychomycosis than normal individuals are, there is little research into the efficacy of standard oral regimens of terbinafine and itraconazole for onychomycosis in the diabetic population. Study design, We present a prospective, randomized, single-blind, parallel group, comparator-controlled, multi-centre study designed to assess the efficacy of the pulse itraconazole (200 mg twice daily, 1 week on, 3 weeks off, for 12 weeks) vs. continuous terbinafine (250 mg once daily for 12 weeks) oral therapies in the treatment of dermatophyte toenail distal and lateral subungual onychomycosis (DLSO) in the diabetic population. Efficacy parameters, Primary efficacy measures included mycological cure rate (negative KOH and culture) and effective cure (mycological cure plus nail plate involvement of 10% or less) at Week 48. Results, At Week 48, mycological cure was attained by 88.2% (30 of 34) and 79.3% (23 of 29) of patients in the itraconazole and terbinafine groups, respectively (P not significant). Effective cure (mycological cure with , 10% of nail plate involvement) was attained by 52.9% (18 of 34) of the itraconazole group and 51.7% (15 of 29) of the terbinafine group (P not significant). Three itraconazole patients experienced side effects in the form of gastrointestinal problems. There were no serious adverse events and no interactions with concomitant medications recorded. Discussion, Both continuous terbinafine and itraconazole pulse therapy are effective and safe in the management of dermatophyte toenail onychomycosis in people with diabetes. [source]

Dexamethasone pulse therapy in pemphigus

Clinical trial: cyclophosphamide pulse therapy , a promising therapeutic alternative in refractory Crohn's disease

ALIMENTARY PHARMACOLOGY & THERAPEUTICS, Issue 12 2009
K. J. SCHMIDT
Summary Background, In severe steroid-refractory Crohn's disease (CD), established therapies fail in a relevant proportion of patients. Recent pilot studies indicated the efficacy of cyclophosphamide pulse therapy in these patients. Aim, To provide further and substantial evidence for the rationale to apply cyclophosphamide pulse therapy as therapeutic option in severe courses of CD. Methods, Fifteen patients with steroid-refractory (n = 13) or steroid-dependent (n = 2) CD received 2,6 (median 3) monthly pulses of 750 mg cyclophosphamide in an open-label fashion. Eleven patients were on concomitant immunosuppression (azathioprine/mercaptopurine n = 9; methotrexate n = 2). Results, Thirteen of 15 patients (87%) had a clinical response (CDAI decrease >100). Ten patients (67%) went into remission (CDAI <150) after 8 weeks. Steroid-free remission was achieved in eight patients (54%). Two patients (13%) failed to respond. Median CDAI decreased from 420 (245,550) to 100 (26,538) at week 8. Remission lasted 16 months (median, range 4,40). In three patients, arthritis, erythema nodosum and episcleritis completely resolved. Cyclophosphamide pulse therapy administration was well tolerated in all subjects. Conclusions, Cyclophosphamide pulse therapy is safe and highly effective for induction and maintenance of remission in steroid-refractory/-dependent CD. There is a strong need for additional experience to improve the setting of the encouraging cyclophosphamide treatment in CD. [source]

Cyclophosphamide pulse therapy followed by azathioprine or methotrexate induces long-term remission in patients with steroid-refractory Crohn's disease

ALIMENTARY PHARMACOLOGY & THERAPEUTICS, Issue 2 2006
C. SCHMIDT
Summary Background In patients with steroid-refractory Crohn's disease, the therapeutic goal is to achieve both rapid remission and maintenance of clinical response. Aim To evaluate the long-term benefit in patients treated with cyclophosphamide pulse therapy and azathioprine or methotrexate, a combination shown to be effective in a recent pilot study. Methods Sixteen patients with acute steroid-refractory Crohn's disease participated in a prospective open-labelled uncontrolled pilot study between December 1998 and June 2003. All had a median number of 4 monthly pulses of intravenous cyclophosphamide (750 mg) and were followed until relapse of the disease. Results Thirteen of 16 patients (81%) achieved remission within 8 weeks after two pulses of cyclophosphamide in combination with azathioprine or methotrexate, with a Crohn's Disease Activity Index decrease from 294 to 111 (median). Remission sustained for 19 months (median, range: 1,45). Moreover, eight patients with pyoderma gangrenosum and erythema nodosum who responded to cyclophosphamide have maintained their remission for up to 30 months. Conclusions In steroid refractory patients with Crohn's disease, cyclophosphamide is highly effective to induce remission. This uncontrolled study indicates that cyclophosphamide-induced remission is long-lasting under standard immunosuppressive therapy. [source]

Treatment of dermatophyte onychomycosis with three pulses of terbinafine (500 mg day,1 for a week)

MYCOSES, Issue 1 2009
Y. Takahata
Summary We assessed the safety and efficacy of pulse therapy with terbinafine tablets in 55 patients with dermatophytic onychomycosis. One pulse consisted of oral terbinafine tablets (500 mg day,1) given for 1 week usually followed by a 3-week interval. This regimen was repeated twice. Topical 1% terbinafine cream was applied daily. Efficacy was assessed based on both clinical and mycological examinations 1 year after treatment initiation. We observed a complete cure in 41 patients (74.5%), marked improved in three patients (5.6%), slight improvement in three patients (5.6%) and drop out in six patients (10.7%). Two patients (3.6%) discontinued terbinafine because of gastrointestinal disturbance (one patient) and drug-induced eruption (one patient). No patient had abnormal laboratory findings, including liver function tests. In summary, a regimen of three pulses of terbinafine therapy given daily for 1 week in combination with topical application of terbinafine cream appears to be safe and effective in treating dermatophytic onychomycosis and offers advantages in convenience and cost-effectiveness compared with continuous dosing. [source]

Cutaneous zygomycosis: a case report and review of Japanese reports

MYCOSES, Issue 7-8 2001
M. Kobayashi
Rhizopus microsporus var. microsporus; Hautinfektion; abwehrgeschwächter Patient. Summary. A 69-year-old man, a carpenter with idiopathic thrombopenic purpura and interstitial pneumonia, was treated with steroid pulse therapy and antibiotics. On the seventh day of steroid therapy, a conglomeration of papules, vesicles and pustules appeared in an area of the left buttock in contact with his napkin. In a Parker KOH specimen of the crust of the lesion, many non-septate hyphae were seen, and culture of material obtained by biopsy yielded Rhizopus microsporus var. microsporus. Ketoconazole cream was applied topically for 1 week, and the exanthema healed. After the third month of in-patient treatment, the patient's overall condition had returned to normal, and he was discharged. Cutaneous zygomycosis is a rare disease, and only 19 cases have been reported in Japan. Its characteristics, as reported in these cases, have been collected and collated. Zusammenfassung., Ein 69 Jahre alter Mann, ein Zimmermann mit idiopathischer thrombopenischer Purpura und interstitieller Pneumonie, begann eine Steroid-Pulstherapie und Antibiotika-Therapie. Am siebten Tag der Steroidgabe bildete sich im Bereich der linken Gesäßhälfte, an der Kontaktstelle mit einer Windel, eine Ansammlung von Papeln, Bläschen und Pusteln. Im Parker-KOH-Präparat von Proben der Blasenkrusten fanden sich zahlreiche nicht-septierte Hyphen, und in Kultur wurde Rhizopus microsporus var. microsporus nachgewiesen. Eine Woche lang wurde lokal Ketoconazol-Creme aufgetragen, und das Exanthem heilte ab. Nach dem dritten Monat der Hospitalisierung normalisierte sich das Befinden des Patienten und er konnte entlassen werden. Die Haut-Zygomykose ist sehr selten, und in Japan wurden bisher nur 19 Fälle berichtet. Diese Kasuistiken wurden gesammelt und ausgewertet. [source]

Clinicopathological studies of peripheral neuropathy in Churg,Strauss syndrome

NEUROPATHOLOGY, Issue 4 2002
Toshiko Nagashima
Clinicopathological studies were performed on the visceral organs and the sural nerve of a male patient with Churg,Strauss syndrome (CSS) in order to understand the mechanisms of peripheral nervous system damage. A 67-year-old man, with a 2-year history of bronchial asthma, developed acutely painful paraplegia and dyspnea. Laboratory data showed a leukocytosis, an elevated serum creatinine kinase (CK) and marked eosionophilia. Autoantibodies including p- and c-ANCA were negative. Electrophysiological studies revealed a severe sensory-motor neuropathy of multiple mononeuritis type. Steroid pulse therapy performed a day after biopsy of skin, muscle and sural nerve was effective in resolving his respiratory and neurological dysfunction but a perforation of an intestinal ulcer occurred which required surgical intervention. In the biopsied sural nerve and the surgically resected intestine and mesentery there was vasculitis with fibrinoid necrosis accompanied by numerous eosinophils and macrophages containing eosinophil cationic protein (ECP). These findings suggest that in addition to ischemic changes due to vasculitis some neurotoxic substances generated by the eosinophils may be involved in the development of neuropathy in CSS. [source]

Systemic granulomatous necrotizing vasculitis in a MPO,ANCA-positive patient

PATHOLOGY INTERNATIONAL, Issue 8 2004
Atsushi Kurata
We present a case of myeloperoxidase antineutrophil cytoplasmic antibody (MPO,ANCA)-associated vasculitis that demonstrated a systemic granulomatous lesion at autopsy. The patient initially showed anorexia, general malaise and anemia. Colon fiber was examined to detect the bleeding site, which revealed ischemic mucosal damage associated with venous fibrin thrombus. Because a high titer of MPO,ANCA was found, ANCA-associated vasculitis was suspected and the patient was started on steroid pulse therapy. However, anemia, renal failure and respiratory failure worsened and the patient died of sudden cardiac failure 2 days after the start of the therapy. An autopsy revealed systemic arteritis in multiple organs including the kidneys, liver, spleen, gastrointestinal system and genital organs that indicated fibrinoid necrosis accompanied by granulomatous reaction with multinucleated giant cells; the granulomatous reaction further extended along the splenic capsule. Glomerulonephritis and diffuse pulmonary damage, which are common in MPO,ANCA-associated vasculitis, were almost absent but parapleural fibrosis was present. The direct cause of death was presumed to be hemorrhagic shock due to rupture of an aneurysm in the gastric subserosa. As far as we know, this is the first case of a systemic granulomatous reaction in MPO,ANCA-positive vasculitis, although the cause of the granulomatous lesion is unknown. [source]

Treatment of allergic alveolitis with methylprednisolone pulse therapy

PEDIATRIC ALLERGY AND IMMUNOLOGY, Issue 1 2003
Christiane Chen
We report on a 13-year-old-boy who had been admitted to our hospital for dyspnea, hypoxia, and pulmonary infiltrates. The diagnosis of allergic alveolitis was based on history (provocation by exposure), lung function tests, bronchoalveolar lavage, and transbronchial lung biopsy. No specific allergen could be identified. Five courses of methylprednisolone pulse therapy (15 mg/kg on three consecutive days) stabilized the patient with normalization of lung function and blood gas analysis. Between pulses the boy returned to his home on a farm without relapse. It is estimated that the effect of a single pulse lasted for at least 2,4 weeks. We conclude that pulse therapy can be used instead of continuous therapy in this rare disease in childhood. [source]

Celiac disease and pulmonary hemosiderosis in a patient with chronic granulomatous disease

PEDIATRIC PULMONOLOGY, Issue 4 2004
Dominik Hartl MD
Abstract We report on a patient with the hitherto undescribed combination of chronic granulomatous disease, pulmonary hemosiderosis, and celiac disease. The hemosiderosis resolved with a gluten-free diet and glucocorticosteroid pulse therapy, but the restrictive lung function pattern remained unchanged. Lung function improved markedly by immunosuppression with daily glucocorticosteroid and azathioprine treatment. Pediatr Pulmonol. 2004; 38:344,348. © 2004 Wiley-Liss, Inc. [source]

Churg-Strauss syndrome revealed by acute abdominal pain

PEDIATRIC PULMONOLOGY, Issue 1 2001
M. Berlioz MD
Abstract We describe a 10-year-old girl with Churg-Strauss syndrome, who presented with acute abdominal pain, bloody diarrhea, and pulmonary infiltrates. She had a 6-year history of severe asthma. Bronchoalveolar lavage showed marked eosinophilia. She responded well to high-dose intravenous corticosteroid pulse therapy for 3 consecutive days, followed by oral steroids without developing major side effects. This case should remind pediatricians of the rare existence of this vasculitis in children. Relapse is not uncommon, and long-term careful supervision is necessary. Pediatr Pulmonol. 2001; 32:92,94. © 2001 Wiley-Liss, Inc. [source]

Cat scratch disease and acute rejection after pediatric renal transplantation

PEDIATRIC TRANSPLANTATION, Issue 4 2002
Vikas R. Dharnidharka
Abstract: Cat scratch disease (CSD) can lead to unexplained fever, generalized lymphadenopathy and organomegaly in immunocompetent individuals. CSD has rarely been reported in immunocompromised transplant recipients, where its clinical features would mimic the more common post-transplant lymphoproliferative disease (PTLD). We report three cases of CSD seen recently in children who had received prior kidney transplants. The three children were between 7 and 9 yr old, and had received kidney transplants 2,4 yr prior, with stable renal function. In each case, there was unexplained fever with either lymphadenopathy or organomegaly. The diagnosis of CSD was suggested by a history of new cats being introduced into each household and confirmed in all cases by the serological presence of a significant titer (> 1,:,64) of IgM antibodies to Bartonella henselae. Tests for other bacterial infections, cytomegalovirus and Epstein,Barr virus infections were negative. All the patients showed a clinical improvement with anti-microbial therapy. In patients A and B, the CSD was associated with an acute rejection episode shortly after diagnosis. The rejection episodes were reversed by intravenous steroid pulse therapy. Only four cases of CSD have been previously reported following solid organ transplantation. Acute rejection following CSD has not been previously reported. CSD should be included in the differential diagnosis of fever in the post-transplant setting, especially where PTLD is suspected. [source]

Mizoribine oral pulse therapy for a patient with polyarticular juvenile idiopathic arthritis

PEDIATRICS INTERNATIONAL, Issue 6 2006
ATSUSHI HAYASHI
No abstract is available for this article. [source]

Efficacy of mizoribine in the treatment of systemic lupus erythematosus in children

PEDIATRICS INTERNATIONAL, Issue 4 2004
Kouichi Yoshidome
AbstractBackground:,Mizoribine (MZR) is a novel immunosuppressant developed in Japan. As MZR is reported to be less toxic than other cytotoxic drugs, it is frequently used in Japan in the treatment of adult patients with rheumatoid arthritis or lupus nephritis. The objective of this study was to evaluate the efficacy of MZR in children with SLE. Nine female children with lupus nephritis who had undergone renal biopsy before starting MZR, were involved in this study. Their mean disease duration was 4.8 years at the time MZR treatment was initiated. Patients who had received intensive medications, such as methyl-prednisolone pulse therapy, intravenous cyclophosphamide pulse therapy, and/or other immunosuppressants, within the 4 months prior to the start of the study, were excluded. Methods:,Patients treated with 3 mg/kg per day of MZR were monitored every month for up to 1 year. The efficacy of MZR was evaluated by the changes from baseline values of serum C3, serum C4, anti-dsDNA antibody titer, erythrocyte sedimentation rate (ESR), urinary protein, dosage of prednisolone (PSL), and the sum of the scores defined by these parameters. Results:,Favorable changes were observed in C3 and ESR after 2 months and 3 months of MZR therapy, respectively. At 3 months of MZR therapy, the sum of scores defined by the parameters for disease activity indicated that MZR was more effective in non-class IV nephritis patients (n = 5) than in class IV nephritis patients (n = 4) (P = 0.0197). All nine children involved in the study tolerated the MZR therapy well during the study. Conclusion:,MZR was safe in lupus children, but its efficacy was limited in patients with non-class IV nephritis. Further study is necessary, in which higher dosages and/or earlier use of MZR is provided to a larger number of children. [source]

Elevated levels of leukotriene B4 and leukotriene E4 in bronchoalveolar lavage fluid from patients with scleroderma lung disease

ARTHRITIS & RHEUMATISM, Issue 6 2003
Otylia Kowal-Bielecka
Objective The leukotrienes are a family of arachidonic acid,derived lipid mediators with proinflammatory and profibrotic properties. The aim of this study was to analyze the role of leukotriene B4 (LTB4) and LTE4 in the pathogenesis of scleroderma lung disease (SLD). Methods Nineteen systemic sclerosis (SSc) patients with SLD, 11 SSc patients without SLD, and 10 healthy controls were studied. Bronchoalveolar lavage (BAL) fluid was obtained during routine bronchoscopy of the right middle lobe in all study subjects. Levels of LTB4 and LTE4 were measured using enzyme immunoassay kits. Results Levels of LTB4 and LTE4 were significantly higher in SSc patients with SLD (251 ± 170 pg/ml and 479 ± 301 pg/ml, respectively), than those in patients without SLD (114 ± 86 and 159 ± 149 pg/ml) and those in normal controls (86 ± 49 and 110 ± 67 pg/ml). In the total group of patients with SSc, levels of both leukotrienes correlated positively with the total number of cells in the BAL fluid and correlated negatively with the forced vital capacity. After intravenous pulse therapy with cyclophosphamide in 6 patients, there was a significant reduction in the concentration of LTB4 (from 380 ± 196 pg/ml to 155 ± 123 pg/ml) but no significant difference in the levels of LTE4 (from 697 ± 325 pg/ml to 418 ± 140 pg/ml). Conclusion Our findings show that LTB4 and LTE4 levels are elevated in SSc patients with SLD and correlate with parameters of inflammation in the lungs. These results indicate that leukotrienes may contribute to the pathogenesis of SLD and may represent a new therapeutic target. [source]

Lithium-induced Hashimoto's encephalopathy: a case report

BIPOLAR DISORDERS, Issue 7 2008
Masanori Nagamine
Objective:, To report on a patient with Hashimoto's encephalopathy induced by lithium. Patient and interventions:, A 61-year-old woman with a type II bipolar disorder and a history of lithium-induced thyrotoxicosis associated with silent thyroiditis was hospitalized to treat a severe major depressive episode. Given long-term treatment with levothyroxine for hypothyroidism that had resulted from silent thyroiditis, endogenous hormone in thyroid follicles was assumed to be minimized by the negative feedback, decreasing risk of recurrent thyrotoxicosis if lithium were restarted. Results:, Lithium clearly relieved the patient's depressive symptoms, but after 40 days encephalopathy developed. Thyrotoxicosis was ruled out, and serum antithyroid antibody titers were elevated. In the cerebrospinal fluid, protein content was substantially elevated and antithyroid antibodies were detected. Encephalopathy resolved dramatically after course of intravenous pulse therapy with methylprednisolone. Conclusions:, We believe that autoantibodies against antigens shared by the thyroid gland and the brain were induced by exposure to lithium, causing the patient to develop Hashimoto's encephalopathy. [source]

The application of temperature measurement of the eyes by digital infrared thermal imaging as a prognostic factor of methylprednisolone pulse therapy for Graves' ophthalmopathy

ACTA OPHTHALMOLOGICA, Issue 5 2010
Shyang-Rong Shih
Abstract. Purpose:, Graves' ophthalmopathy (GO) involves autoimmune process resulting in proptosis, congestion, oedema and diplopia. Werner's NOSPECS classification and clinical activity score (CAS) of GO cannot objectively describe the inflammatory status. Digital infrared thermal imaging (DITI) detects local temperature and may reflect the degree of orbital inflammation. The aim of this study was to evaluate the clinical application of the eye temperature measured by DITI. Methods:, Forty-six patients with GO receiving intravenously methylprednisolone pulse therapy (MPT) were included in this study. Local temperatures of the lateral orbit, upper eyelid, inner caruncle, medial conjunctiva, lateral conjunctiva, lower eyelid and cornea were measured with DITI before and after MPT. CAS, proptosis, eye movement (EOM) and diplopia were also recorded. Improvement of CAS was defined as at least one point decrease at either side of the eye, which was 0.5 score decrease as to the average of bilateral CAS. Results:, Local temperatures of the eyes decreased after MPT. The mean value of temperature (MT) of 12 points including the lateral orbit, upper eyelid, inner caruncle, medial conjunctiva, lateral conjunctiva and lower eyelid of both eyes before MPT was 32.65°. The mean change of MT after MPT (,T) was ,0.22°. ,T significantly negative-correlated with basal MT (correlation coefficient = ,0.54, p = 0.004). Higher baseline MT and CAS before MPT correlated with higher possibility of improvement of CAS after MPT (p = 0.013 and 0.012, respectively). Baseline MT and CAS together correlated with improvement of CAS after MPT better than baseline CAS alone could do (area under the receiver operating characteristic curve: 82.81% and 66.63%, respectively). Conclusions:, Basal temperature of the eyes measured by DITI was an objective indicator of inflammation of GO. Combining CAS and MT could better predict the outcome of MPT than CAS alone. [source]

Systemic lupus erythematosus-associated optic neuritis: clinical experience and literature review

ACTA OPHTHALMOLOGICA, Issue 2 2009
Yen-Ching Lin
Abstract. Purpose:, This study aimed to evaluate the visual outcome of optic neuritis in patients with systemic lupus erythematosus (SLE). Methods:, A retrospective, case-observational study was conducted by reviewing eight patients with SLE-associated optic neuritis from January 1986 to October 2004. The demographic data, clinical manifestations, laboratory data, medical management and disease courses of these eight patients were retrospectively analysed. Main outcome measurements included final visual acuity (VA) and relapse of optic neuritis. Statistical analyses were made using the chi-square test and a linear regression model. The English-language literature on SLE-associated optic neuritis was reviewed. Results:, Initial visual loss was severe in SLE-associated optic neuritis. Seven patients (87%) had VA < 20/200 at onset. All patients received steroid pulse therapy followed by oral steroid tapering. Final visual outcome was highly variable, ranging from the complete recovery of VA in four patients, to partial recovery in one and poor recovery in three. Better visual recovery occurred in patients who received earlier treatment (within 10 days). However, longer duration of steroid administration was found to have no significant benefit on visual outcome. Conclusions:, Systemic lupus erythematosus-associated optic neuritis is not common. However, it is important that ophthalmologists differentiate SLE-associated optic neuritis from idiopathic optic neuritis because of the severe visual impairment and steroid dependence associated with the former. Early diagnosis and prompt treatment are important for restoring visual function in these patients. [source]

ABO-incompatible renal transplantation in Epstein syndrome

CLINICAL TRANSPLANTATION, Issue 2010
Masao Ogura
Ogura M, Kikuchi E, Kaito H, Kamei K, Matsuoka K, Tanaka H, Kuroda T, Sekine T, Ito S. ABO-incompatible renal transplantation in Epstein syndrome. Clin Transplant 2010: 24 (Suppl. 22): 31,34. © 2010 John Wiley & Sons A/S. Abstract:, Epstein syndrome (ES) is an autosomal dominant hereditary disease characterized by hereditary nephritis, sensory deafness, and thrombocytopenia. We herein report the case of a 20-yr-old man with ES who underwent ABO blood type-incompatible living-donor kidney transplantation from his mother. He was given platelet transfusion, and his pre-operative number of platelets were 108 × 103/,L. After transplantation, urine output and the decrease in serum creatinine (sCr) were within the acceptable ranges. On the seventh post-operative day (POD), sCr had risen and urine output decreased. Anti-type A antibody rapidly elevated from <2 times (×2) just before transplantation to 64 times (×64), and the patient required hemodialysis again. Resistance index (RI) by ultrasound increased from an average of 0.5 , 0.6 on POD 1 to an average of 0.7 , 0.8 on POD 7. However, several biopsies (POD 4, 7, and 10) showed no obvious findings of acute rejection except for intense C4d deposition. Because acute antibody-mediated rejection was not completely ruled out, he was treated with methyl-prednisolone pulse therapy, plasma exchange, cyclophosphamide, and immunoglobulin. Regardless, his titer of anti-type A antibody was still high, and he still presented oliguria. We performed an emergent splenectomy. Consequently, the levels of anti-type A antibody decreased, the RI also dropped to an average of 0.6. However, on POD 19 and 25 (platelets were 27 × 103/,L and 36 × 103/,L), he developed a massive intraperitoneal hematoma around the graft and region of the removed spleen, which pushed the graft out and caused acute tubular necrosis, resulting in anuria. The RI rose to an average of 0.8 , 1.0 after these episodes. He also experienced bleeding from a duodenal ulcer on POD 21. However, his renal function has fully recovered after acute hemodialysis for 35 d. The latest sCr was 1.5 mg/dL with a recovery in RI to 0.6. Although his platelet count was maintained at a minimum of 50 × 103/,L, he had several severe bleeding episodes, concluding that sufficient platelets are necessary after transplantation in ES. [source]

An ABO-incompatible renal transplant patient who developed severe antibody-mediated vascular rejection 36 days after transplantation

CLINICAL TRANSPLANTATION, Issue 2008
Akiko Fujii
Abstract:, A 44-yr-old man had an ABO-incompatible renal transplantation from his 41-yr-old wife. He was diagnosed with IgA nephropathy at the age of 31 and began hemodialysis to treat chronic renal failure at the age of 39. Preoperative flow panel reactive antibody was negative. An episode biopsy on post-operative day (POD) 18 showed mild infiltrative vasculopathy (v1, g2, ptc1, TMA, PTC+). The serum creatinine (sCr) was 2.26 mg/dL. Anti-A antibody was x32. Double filtration plasmapheresis and plasma exchange were performed. A second episode biopsy was performed on POD 36. The Cr was 3.73 mg/dL. Anti-A antibody was x32. Histologically, antibody-mediated vascular rejection with severe fibrinoid necrosis in the lobular arteries was detected (v3, g2, ptc2, TMA, PTC+). Steroid pulse therapy was performed, and OKT-3 was administered for 10 d. The anti-A antibody titer was x128 on POD 47. A biopsy specimen obtained on POD 55 showed severe vascular rejection with stenosis of the vascular lumen and fibrinoid necrosis (v3, cv2, g1, cg2, ptc1, TMA, PTC+). The sCr was 7.09 mg/dL. Despite double-filtration plasmapheresis, the patient was reintroduced to hemodialysis. Here, we report a case showing the typical histological features of antibody-mediated vascular rejection. [source]

Successful rescue and maintenance of long-term remission of anti-HLA antibody-mediated acute allograft rejection by rituximab-containing therapy: case report

CLINICAL TRANSPLANTATION, Issue 2008
Yasuo Takeuchi
Abstract:, A 38-yr-old man with chronic renal failure received a second kidney transplantation from a cadaveric donor. Complement-dependent cytotoxicity cross-match (CDC) was negative against T cells, but positive with the B-cell warm test. Human leukocyte antigen (HLA)-typing showed a one haplo-identical match. The blood type was compatible. He was treated with tacrolimus, mycophenolate mofetil (MMF), methylpredonisolone (MP), and basiliximab as immunosuppressive therapy. A clinical episode graft biopsy and Flow-PRA on post-operative day (POD) 19 showed anti-HLA antibody-mediated acute rejection (AHR). The patient was treated with plasmapheresis (PP). Renal biopsy performed on POD 65 because of re-rise of serum creatinine level showed worsening of renal injury. The patient was treated with rituximab (100 mg/body) with PP and MP pulse therapy, followed by tacrolimus and MMF. Graft function thereafter improved. A renal allograft biopsy specimen on POD 300 and Flow-PRA showed the remission of AHR within one-yr after transplantation. [source]