Effect and safety of methylprednisolone pulse therapy on spinal nerve function in patients with acute spinal cord injury / 中国生化药物杂志

Objective To investigate the effect of methylprednisolone pulse therapy on spinal nerve functions in patients with acute spinal cord injury (ASCI) and evaluate its safety. Methods 200 patients with ASCI treated in our hospital from January 2010 to December 2016 were selected and were randomly divided into two groups,with 100 cases each group. The patients in control group were treated with routine basic treatment while patients in the study group were treated with methylprednisolone pulse therapy. On the basis of the American spinal cord injury Society scoring criteria (ASIA), the neurological functional recovery scores were performed were scored before and 6 weeks after treatment between the two groups and the adverse reactions were recorded. Results The scores of sensory and motor function of the patients in the two groups were higher than those before the treatment and there was statistical difference (P<0.05), and the scores in the study group was significantly higher than those in the control group, and the difference between the two grouPs was statistically significant (P<0.05). The incidence of adverse reactions in the study group (25.00％) was significantly higher than that in the control group (10.00％), and the difference was statistically significant (P<0.05). The incidence of lung infection and gastrointestinal reaction in the study group were significantly higher than those in the control group and there were statistical difference (P<0.05). Conclusion Methylprednisolone pulse therapy has significant effect on the recovery of neurological function in patients with ASCI, but the incidence of adverse reactions is high, so it is necessary to strengthen the monitoring and intervention in clinic.

Hypokalemia-induced Polyuria with Nocturia after Intravenous Methylprednisolone Pulse Therapy in a Henoch-Schonlein Purpura Nephritis Patient

Patients with moderate to severe degrees of Henoch-Schonlein purpura (HSP) nephritis receive high-dose intravenous methylprednisolone pulse therapy (IMPT). Although the regimen is generally safe and effective, various complications occasionally develop. administration of excessive corticosteroid can induce urinary potassium wasting leading to hypokalemia. Polyuria, one of the complications of hypokalemia, is related to both increased thirst and mild nephrogenic diabetes insipidus. And hypokalemia itself also impairs the maximal renal urinary concentration ability. Although polyuria or nocturia after IMPT is not common, it is correctable immediately by oral potassium supplementation. Therefore, during IMPT, careful history taking of nocturia as well as monitoring urine volume, serum and urine potassium level at regular follow-up are necessary because even mild hypokalemia can provoke urine concentrating ability defect. We experienced a case of 11 year-old boy with HSP nephritis who suffered from hypokalemia-induced polyuria with nocturia right after IMPT.

A Case of Giant Cell Granulomatous Hypophysitis with Recurrent Hypoosmolar Hyponatremia

A 39-year-old woman presented with a 20 day history of recurrent hypoosmolar hyponatremia. Because her volume status seemed to be normal, the most suspected causes of her hyponatremia were adrenal insufficiency and hypothyroidism. Endocrinologic examination, including a combined pituitary function test, showed TSH and ACTH deficiency without GH deficiency, and hyperprolactinemia was also present. Sella MRI showed a pituitary mass, stalk thickening and loss of the normal neurohypophysial hyperintense signal on the T1 weighted image. Pathologic exam demonstrated granulomatous lesions and Langhans' multinucleated giant cells with inflammatory cell infiltration. After high dose methylprednisolone pulse therapy (1 g/day for 3 days) with subsequent prednisolone and levothyoxine replacement, there was no more recurrence of the hyponatremia. The sella MRI on the 6th month showed decreased mass size, narrowed stalk thickening and the reappearance of the normal neurohyphophysial hyperintense signal. She is currently in a good general condition and is receiving hormone replacement therapy.

The Prognosis of Focal Segmental Glomerulosclerosis Patients with Methylprednisolone Pulse Therapy Alone

PURPOSE: Since the first report by Mendoza in 1990, there have been several studies reporting that long-term intravenous methylprednisolone(MP) pulse therapy combined with cyclosporin A(CsA) or cyclophosphamide might be beneficial for the treatment of steroid resistant focal segmental glomerulosclerosis(FSGS). We investigated the therapeutic effect of long-term MP pulse therapy without CsA or cyclophosphamide on steroid resistant FSGS. METHODS: The medical records of the 10 steroid resistant FSGS patients who were treated with MP pulse therapy by the Mendoza protocol without CsA or cyclophosphamide in our hospital were retrospectively reviewed. RESULTS: The median age at onset was 2.6 years(range 1.1-10.6 years) and the median age at the initiation of therapy was 5.7 years(range 1.8-20 years). The median duration of follow-up was 35 months(range 4-132 months). At the end of therapy, 5 patients achieved complete remission(50%) and 2 partial remission(20%), one of whom relapsed after the therapy. Three patients did not respond to the therapy, two of whom progressed to end-stage renal failure during the therapy eventually requiring kidney transplantation. CONCLUSION: Intravenous long-term MP pulse therapy without CsA or cyclophosphamide by the Mendoza protocol may be effective in a subset of patients with steroid-resistant FSGS.

Histopathologic Diagnosis and Outcome of Renal Biopsied Pediatric Nephrotic Syndrome

PURPOSE: To determine the histological findings and treatment outcome in cases of childhood nephrotic syndrome which required renal biopsy. METHODS: We retrospectively reviewed the clinical, laboratory, pathologic findings and therapeutic outcomes of 169 nephrotic children who received a renal biopsy at the Department of Pediatrics, Kyunghee Medical University Hospital, Seoul from 1984 to 2004 over a period of 21 years. The renal biopsy was performed in nephrotic children who showed atypical features at presentation, or needed cytotoxic therapy because of frequent-relapsing, steroid-dependent, or steroid-resistant nephrotic syndrome(SRNS). RESULTS: Minimal change disease(MCD) was found in 52.1% of the patients, followed by diffuse mesangial proliferation(33.1%), focal segmental gomerulosclerosis(5.3%), membranoproliferative glomerulonephritis(2.4%), membranous nephropathy(2.4%), and IgA nephropathy(1.8 %). In MCD children, 14.8% had hematuria, 22.7% had hypertension, 5.7% showed decreased renal function, and no patient was found to have an abnormal complement level. Among patients diagnosed with diseases other than MCD, 43.2% had hematuria, 21.0% was found to be hypertensive, 7.4% of children showed decreased renal function and only 3(3.7%) had decreased complement level; the rates of hematuria and SRNS were found to be significantly higher than MCD patients. Among 37 SRNS patients, 30(81.0%) showed a final remission state with long-term steroid therapy, including methylprednisolone pulse therapy, over 4 months, with or without cytotoxic therapy. CONCLUSION: Almost half of the cases of childhood nephrotic syndrome requiring renal biopsy were not diagnosed with MCD. Among atypical features, hematuria and steroid-resistance would be the most probable indicators for a diagnosis other than MCD. Even in patients with SRNS, long-term methylprednisolone pulse therapy may result in a good remission rate.

A Case of Systemic Lupus Erythematosus Presenting with Protein Losing Enteropathy / 대한류마티스학회지

Protein losing enteropathy (PLE) is characterized by the loss of protein into the gastrointestinal tract that results in hypoalbuminemia and generalized edema. PLE is associated with several clinical disorders, but it is a rare manifestation of systemic lupus erythematosus (SLE), and it may be the presenting manifestation of SLE. We report a patient with SLE presenting with PLE, in whom methylprednisolone pulse therapy was highly effective. A 29-year-old women was admitted to our hospital with generalized edema. Laboratory findings revealed hypoalbuminemia, hypercholesterolemia and antinuclear antibody 1:160, speckled type. Mucosal biopsies of the duodenal bulb and terminal ileum revealed edema and dilated lymphatics with infiltration of chronic inflammatory cells. PLE was diagnosed by marked elevation of alpha-1 antitrypsin clearance in stool and abnormal radioactivity within small intestine on 99mTc-labeled human serum albumin scan. Hypoalbuminemia and generalized edema improved rapidly after methylprednisolone pulse therapy.

High-dose Methylprednisolone Pulse Therapy in Measles Postinfectious Encephalomyelitis: Two Cases of Successful Recovery / 대한소아신경학회지

We treated two patients of measles postinfectious encephalomyelitis with intravenous methylprednisolone pulse therapy(1 g/1.73 m2/day for 5 days) 38 hours and 5 days respectively after the onset of neurologic symptoms. Despite extensive white matter involvement shown in MRI and severe clinical symptoms, the patients recovered from their neurologic symptoms dramatically following intravenous methylprednisolone pulse therapy. Because acute postinfectious encephalomyelitis has been postulated to be immunologically mediated disease, instead of direct viral invasion, ACTH and dexamethasone are widely used and the outcome is variable. This case report of successful recovery from fulminant ADEM with pulse intravenous methylprednisolone therapy, although uncontrolled, suggests that this therapeutic regimen should be studied in other cases.

Infantile hemangioendothelioma treated with high dose methylprednisolone pulse therapy

Infantile hemangioendothelioma is a severe disease with a high mortality. It is characterized by multiple hemangioma affecting the skin and visceral organs. We report that high doses of methylprednisolone pulse therapy improved symptoms and signs of infantile hemangioendothelioma in a male neonate, and completely resolved the hepatic and cutaneous hemangioendothelioma on follow up.

A Case of Persistent Kawasaki Disease Improved with Pulsed Doses of Methylprednisolone / 소아알레르기및호흡기학회지

Kawasaki disease is an acute vasculitis of unknown causes that occurs predominantly in infants and young children and produces coronary artery aneurysm. We have recently experienced a case of persistent Kawasaki disease in a 24 month-old-girl improved with pulsed doses of methylprednisolone. Even with an administration of intravenous gamma globulin(IVIG), she repeated the course of recovery and aggravation. After four times repeated doses of IVIG, additional intravenous methylprednisolone pulse therapy was tried and resulted in remarkable improvement. We reported the case with a brief review of the related literature.

The Effects of Intravenous Methylprednisolone Pulse Therapy by Mendoza Protocol in Primary and Secondary Nephrotic Syndrome

PURPOSE: Since Mendoza(1990)'s report that long term methylprednisolone pulse therapy by Mendoza protocol (MP therapy) is a good treatment option in focal segmental glomerulo -sclerosis(FSGS), there have been reports of the effects of this therapy in steroid-resistant nephrotic syndrome. However, no studies have been performed on the effects of MP therapy in steroid-dependent nephrotic syndrome and secondary nephrotic syndrome. In this study, we investigated the effects of long term MP therapy in primary and secondary nephrotic syndrome in which previous treatment options were not effective. METHODS: We chose 10 children who were diagnosed with steroid-dependent minimal change nephrotic syndrome(SD-MCNS), who had shown frequent relapse during the immunocompromised or cytotoxic therapy period, and 6 children with FSGS and 5 children with secondary nephrotic syndrome children, who had shown no response during the previous therapy period. We treated these patients according to Mendoza protocol involving infusions of high doses of methyl- prednisolone, often in combination with oral cyclophosphamide for 82 weeks. RESULTS: In all the 10 children with SD-MCNS, complete remission was visible on average of 18+/- days after MP therapy was started. However, all these children relapsed during or after MP therapy. In these children, the mean relapse rate prior to MP therapy was 2.1+/-.0 relpases/year, which was reduced to 1.4+/-.9 relapses/year during MP therapy(P>0.05) and rose to 2.7+/-.0 relapse/year after MP therapy. Of the 6 children with FSGS, 4 children(67%) showed complete remission, of whom 3 children(50%) remained in the remission status during the follow up period, 1.2+/-.7 years, after the end of MP therapy. 2 children(33%) showed no response. All of the 5 children with secondary nephrotic syndrome showed remission and remained in the remissiom status during the follow up period, 1.7+/-.6 years. The only side effect of MP therapy was transient hypertension in 10 children of all subjects during the intravenous infusion of methylprednisolone. CONCLUSION: We conclude that although long term MP therapy is not effective in the treatment of SD-MCNS, it is an effective therapy against intractable FSGS and secondary nephrotic syndrome.

Immediate Effects of Methylprednisolone Pulse Therapy on the Immune Response

To study the immediate effects of high-dose intravenous methylprednisolone pulse therapy on the immune mechanisms in the absence of other immunosuppressive agents, we treated ten patients with active systemic lupus erythematosus, six with renal and three with central nervous system involvement with three daily 1 gram intravenous doses of methylprednisolone and measured the immune response before and just after discontinuation of the drugs. After the treatment, the mean seum IgG, IgA and IgM levels were essentially unchanged. Likewise, serum C3 and C4 levels were not changed significantly. In nine of ten patients, methylprednisolone pulse therapy reduced the levels of circulating immune complexes (p < 0.05). Thus the immediate clinical improvements with methylprednisolone pulse therapy are suggested to be the result of depression of the circulating immune complex levels.