Casuistic Use of High-Dose Methylprednisolone in a Child with Acute Encephalopathy due to Metabolic Crisis in HIBCH Deficiency
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Abstract 3-hydroxyisobutyryl-CoA hydrolase deficiency (HIBCHD) is a rare metabolic disease. Early symptoms include poor feeding, seizures, hypotonia and impaired psychomotor development. Acute metabolic crisis can cause encephalopathy with high risk of neurological sequelae or death. Casuistic, we here report a nine-year old Danish girl with HIBCHD treated with intravenous high-dose methylprednisolone when presenting with encephalopathy during acute metabolic crisis. Presentation of acute encephalopathy with basal ganglia changes seen on MRI was regarded as acute disseminated encephalomyelitis (ADEM) leading to intravenous high-dose methylprednisolone treatment. The effect of methylprednisolone was profound, not only on the acute neurological symptoms, but also accelerated the development of the child. After re-evaluation of MR images, Whole Genome Sequencing (WGS) confirmed the diagnosis HIBCHD. The high-dose methylprednisolone treatment regime was repeated in a following severe metabolic crisis presenting with acute encephalopathy, dystonia and spasticity. The child survived and after rehabilitation neurological sequelae are present but considerably reduced. We consider if high-dose methylprednisolone should be recommended in children with acute metabolic crisis and encephalopathy due to HIBCHD. Since influenza A virus was the triggering cause to metabolic crisis with encephalopathy, vaccination should be considered in HIBCHD.The apoptosis-inducing effects of i.v. methylprednisolone were investigated as a possible method of controlling inflammation in the CNS in adoptive transfer-experimental autoimmune encephalomyelitis (AT-EAE) in Lewis rats. Two pulses of methylprednisolone were given at the peak of mild and of severe disease. T-cell apoptosis was assessed on spinal cord cross-sections by morphology and TUNEL staining. Concentrations of methylprednisolone were measured in serum, CSF and spinal cord tissue by high-pressure liquid chromatography (HPLC). In severe EAE, 10 mg/kg methylprednisolone increased T-cell apoptosis significantly and T-cell infiltration was marginally decreased. A maximal dose of 50 mg/kg methylprednisolone was superior in both respects and, in contrast to 10 mg/kg methylprednisolone, was also effective in mild EAE. A dose of 1 mg/kg methylprednisolone did not produce notable changes compared with controls treated with phosphate-buffered saline. Serum, CSF and spinal cord concentrations of methylprednisolone measured by HPLC 2 h after a single i.v. injection of 10 or 50 mg/kg methylprednisolone revealed significantly higher methylprednisolone concentrations in severe EAE compared with mild disease. With 50 mg/kg methylprednisolone, we obtained serum and CSF concentrations in the region of 10–5 M methylprednisolone. We also studied the expression of bcl-2, a typical anti-apoptotic regulatory protein, in T cells, and found no change after methylprednisolone treatment compared with controls. Methylprednisolone did not induce apoptosis of oligodendrocytes, which would have been an unwanted side effect in CNS cells. This study provides evidence that methylprednisolone dose-dependently augments T-cell apoptosisin situ in AT-EAE. Our results may have implications for the use of glucocorticosteroids at very high doses in the treatment of inflammatory disorders of the CNS, such as multiple sclerosis, or of other target organs.
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Objective:To evaluate the effect of intrathecal injection of methylprednisolone in patients of acute multiple sclerosis(MS).Methods:Seventy cases were divided into intrathecal methylprednisolone group(n=32) and methylprednisolone impact group(n=38).The reduction of Kurtzke expanded disability status scale(EDSS) and the rate of side effects after treatment were evaluated.The average total cost of hospitalization,the average cost per hospital day and the proportional contribution of medications were analysed with the software of SPSS13.0.Results:The average improvement of EDSS after 5 days treatment was significantly obvious in intrathecal methylprednisolone group compared with that of methylprednisolone impact group(P 0.05),but no difference after 15 days treatment(P 0.05).There were significantly lower values in the average total cost of hospitalization,the average cost per hospital day,average hospitalization day and the proportional contribution of medications in intrathecal methylprednisolone group than those of methylprednisolone impact group(P 0.05).Conclusion:The intrathecal methylprednisolone therapy showed a fast improvement of EDSS scale,reduced average hospitalization day and lower average total cost of hospitalization in patients with multiple sclerosis.
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Objective: The establishment of animal models of septic shock in rabbits, given methylprednisolone (MP) treatment and evaluated its efficacy. Methods: 30 healthy rabbits were randomly divided into methylprednisolone 3 mg/ (kg·d) treatment group, methylprednisolone 1 mg/(kg·d) treatment group,after the shocking, delayed to give methylprednisolone 3 mg/(kg·d) time group and control group, monitored blood pressure in each group pick-up time, cycle time, improving the time and in a stable condition. Results: The recovery time of blood pressure in high-dose methylprednisolone treatment group was shorter than the control group, and the total effective rate were significantly different ( P0.05). Conclusion: In experiment, high-dose methylprednisolone is a safe and effective treatment of septic shock.It may be applied to clinical according to further experiment and clinical study.
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Effects of varying doses of troleandomycin (TAO) on methylprednisolone disposition were examined in five steroid-dependent asthmatic patients. The characteristic reduction in methylprednisolone elimination in the presence of TAO after a 40 mg IV methylprednisolone was also present after methylprednisolone doses as low as 4 mg. In patients receiving continuous TAO on an every-other-day basis, inhibition of methylprednisolone elimination was impaired to a greater extent on the "day on" TAO than on the "day off" TAO Methylprednisolone elimination on the day off TAO was still slower than that before TAO, however, TAO on a multiple-dose schedule resulted in greater reduction of methylprednisolone elimination than after a single TAO dose. These results suggest that TAO induces immediate and continued inhibition of methylprednisolone disposition.
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Objective:To evaluate the clinical course,effect,and drug economy sequential therapy on the patients of COPD-AE with methylprednisolone.Method:We adopted random,parallel,open,control multicentre study.The 116 patients of severe COPD-AE were separated into A,B,and C groups.Methylprednisolone was given 80 mg,VD.,b.i.d. in A and B groups.After 4 days,8 mg of methylprednisolone table was given,P.O.,b.i.d.for 5 days in A group,10 days in B group.16 mg of methylprednisolone table was given,P.O.,b.i.d.from 1st to 14 th day.The antibiotics,bronchodilators,and apophlegmatisant were given routinely.Result:The symptomatic score,FEV 1,FEV 1%,PaO\-2 and PaCO\-2 improved contrasting to pre-treatment.However three groups was no significant difference.The FVC increased markedly in A and B group at the middle and end of treatment.The improved rates of A,B,and C group were 51.5%,48.4%,and 39.4% respectively.The improvement of only cough was faster in A and B group than in C group though the effect of symptoms and signs at 2 nd after treatment in A and B group.The clinical cost was 3723.3 Yuan in A group,markedly less than 5494 Yuan in B group and 5041.1 Yuan in C group.Conclusion:The method of methylprednisolone sequential therapy for 9 days was worth to be extended because of the similar effect among methylprednisolone sequential therapy for 9 days,methylprednisolone sequential therapy for 14 days,and oral administration 14 days,faster effect,and less clinical cost.
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Twelve patients with Graves' ophthalmopathy (grade 1-6, ATA classification) were treated with Cyclosporin-A, systemically in combination with methylprednisolone. We observed slight or moderate favourable effect in 9 cases. Our data suggest that the benefit was due to the methylprednisolone, the effectivity of which was enhanced by the cyclosporin even in the glucocorticoid-resistant cases.
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Neurological dysfunction in AIDS is common, occurring in as many as eighty percent of children. Thus, it is important to recognize the central nervous system imaging appearance of HIV, in particular those of HIV encephalopathy, as this is an AIDS defining illness and with distinct neuro-imaging features essential for early diagnosis and timely therapeutic interventionTo identify the clinical features in HIV-1 infection of the central nervous system and their associated neuroradiological correlates.Retrospective review of the records of all children with HIV-1 encephalopathy identified among children with neurological and developmental problems and who were on follow up at a child development and neurology clinic in an African city.A total of 22 children (10 male and 12 female) with HIV-1 encephalopathy were identified among 2382 children with various forms of neurological and developmental problems and who were on follow up at a child development and neurology clinic for a little bit over eight years period. All the children acquired the infection vertically. The age range of these children was between 10 months to 14 years. The median age was 5.6 years. The mean duration of symptom was 3.2 years. Global delay or regression in development along with signs of pyramidal tract involvement and seizures were the commonest clinical signs observed in these children. Neuro-behavioral problems were commonly observed among preschool and school aged children. In older children and preadolescents focal seizures with or with out neurologic deficit and neuroradiological findings were common. Nonhemorrhagic stroke was rare and occurred in one child and another child had cortical blindness. Three children had no neurological deficit. Rapid progression of the disease carried grave prognosis. Opportunistic infections and tumors of the central nervous system were also uncommon among these children. Brain volume loss with dilatation of the lateral ventricle, bilateral symmetrical or asymmetrical calcification of the basal ganglia and periventricular involvement of the white matter were the commonest neuro-radiological findings observed in these children.Atrophy of the brain with dilatation of the lateral ventricles and calcification of the basal ganglia and peri-ventricular involvement of the white matter were the commonest neuro-radiological findings in children with HIV-1 encephalopathy. Similarly global delay or regression in development along with pyramidal tract signs and seizures were the commonest neurological findings. Behavioral problems were common in preschool and school aged children. Focal seizures were common in older children and preadolescents. Rapid progression of the disease carried grave prognosis.
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Background: Idiopathic thrombocytopenic purpura (ITP) is a rare and autoimmune disorder determined by an abnormal reduction in the number of platelets. The current study aims to evaluate the oxidative stress status of children with ITP in two treatment methods using methylprednisolone and methylprednisolone with intravenous immunoglobulin (IVIG). Materials and Methods: This retrospective study was conducted on 60 children with ITP who referred to Baghaei Hospital in Ahvaz in 2021. All the ITP children were equally divided into two groups, 30 receiving methylprednisolone and 30 receiving methylprednisolone and IVIG. The sampling of the patients’ blood was done in two stages before and after the start of treatment. Then, malondialdehyde (MDA), superoxide dismutase (SOD), total antioxidant status (TAS), total oxidative status (TOS), catalase (CAT) and glutathione were measured according to the instructions in the commercial kit. The analyses were performed using SPSS software version 23. P value < 0.05 was significant. Results: The number of platelets after treatment in methylprednisolone and methylprednisolone+ IVIG groups was 133.44 ± 18.93 and 158.76 ± 34.76 (×103/µL), respectively. Itas significantly increased compared to that before the treatment (P = 0.04). The amount of TAC in the group receiving methylprednisolone + IVIG and the methylprednisolone group was 1.64 ± 0.18 and 1.26 ± 0.53 nm, respectively; there was a remarkable difference between the two groups (P = 0.001). Also, SOD, CAT and glutathione in the methylprednisolone + IVIG group were remarkably higher than those in the methylprednisolone group (P < 0.05). Finally, the levels of TOS were lower in the methylprednisolone + IVIG group (19.74 ± 9.93 μmol) than in the methylprednisolone group (26.65 ± 10.64 μmol) (P = 0.01). Conclusion: A combination of IVIG and methylprednisolone was found to have a greater effect on improving antioxidant status and decreasing the oxidative stress indices of ITP children.
Malondialdehyde
Thrombocytopenic purpura
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A randomised comparison is made between methylprednisolone, 1 g intravenously daily for 7 days, and a standard ACTH regime for the treatment of multiple sclerosis in acute relapse. It is found that methylprednisolone produces a more rapid clinical improvement than ACTH but confers no longer term benefit when the two treatments are compared at 3 months. It is proposed that intravenous methylprednisolone does have a role to play in the management of a patient with an acute relapse of multiple sclerosis.
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