Corticotropin and Cortisone in Management of the Nephrotic Syndrome in Children

Riley, Conrad M.

doi:10.1001/jama.1952.03680130020005

Cited by 15 publications

(4 citation statements)

References 6 publications

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“…In 1951 Luetscher, Deming and Johnson reported the elimination of oedema in 14 patients after treatment with A.C.T.H. ; Riley (1952) remissions after A.C.T.H. and cortisone but stated that they were short-lived and that recurrences were frequent.…”

Section: Treatmentmentioning

confidence: 99%

The Natural History of Nephrosis

Rm¹

1957

Archives of Disease in Childhood

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Section: Treatmentmentioning

confidence: 99%

The Natural History of Nephrosis

Rm¹

1957

Archives of Disease in Childhood

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“…Short-term ACTH treatment results for diuresis response and proteinuria are summarized in Table 1 [ 10 , 14 , 31 – 37 ]. ACTH dose ranged from 20 mg/day to 160 mg/day, with one protocol using the regimen of 150–200 mg/m 2 /day [ 35 ].…”

Section: Resultsmentioning

confidence: 99%

“…Diuresis with edema resolution was the key clinical outcome in the earliest studies of short-term ACTH and ACTH dose was quickly tapered or discontinued following diuresis onset [ 14 , 37 ]. The nine short-term ACTH treatment studies reported diuresis with edema resolution in 74 % (265/356) of patients/treatment courses [ 14 , 31 – 36 ].…”

Section: Resultsmentioning

confidence: 99%

Adrenocorticotropic hormone therapy for the treatment of idiopathic nephrotic syndrome in children and young adults: a systematic review of early clinical studies with contemporary relevance

Lieberman

Pavlova-Wolf

2016

J Nephrol

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Adrenocorticotropic hormone (ACTH) as a treatment for proteinuria due to nephrotic syndrome (NS) has re-emerged over the last decade. Current clinical data are primarily limited to adults with treatment-resistant NS. Largely unknown to today’s clinicians is the existence of early clinical studies, following ACTH’s introduction in the late 1940s, showing sustained proteinuria response in idiopathic NS in predominantly pediatric, treatment-naïve patients. Before ACTH, patients suffered severe edema and high mortality rates with no reliable or safe treatment. ACTH dramatically altered NS management, initially through recognition of diuresis effects and then through sustained proteinuria remission. This review synthesizes early clinical literature to inform current NS patient management. We undertook a MEDLINE search using MeSH terms “adrenocorticotropic hormone” and “nephrotic syndrome,” with limits 1945–1965 and English. Sixty papers totaling 1137 patients were found; 14 studies (9 short-term, five long-term, N = 419 patients) met inclusion criteria. Studies were divided into two groups: short-term (≤28 days) and long-term (>5 weeks; short-term initial daily treatment followed by long-term intermittent)ACTH therapy and results were aggregated. An initial response, defined as a diuresis, occurred in 74 % of patients/treatment courses across nine short-term ACTH studies. Analyzed in eight of these studies, proteinuria response occurred in 56 % of patients/treatment courses. Across five long-term ACTH studies, proteinuria response was shown in 71 % of patients and was sustained up to 4.7 years following treatment. The inventory and re-evaluation of early clinical data broadens the evidence base of clinical experiences with ACTH for implementation of current treatment strategies and aiding the design of future studies.Electronic supplementary materialThe online version of this article (doi:10.1007/s40620-016-0308-3) contains supplementary material, which is available to authorized users.

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“…Adrenocorticotropic hormone (ACTH) is a pituitary polypeptide hormone that stimulates the adrenal glands to generate cortisol. ACTH was widely used to treat childhood nephrosis in the United States in the 1950s and was shown to be effective in ameliorating proteinuria and in improving patient survival ( 6 ). These effects of ACTH depended on the dose and duration of ACTH treatment.…”

Section: Introductionmentioning

confidence: 99%

Real-word adrenocorticotropic hormone treatment for childhood-onset nephrotic syndrome

Wang

Dang²,

Wu³

et al. 2023

Front. Pediatr.

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BackgroundCurrent first-line anti-proteinuric treatments do not produce a satisfactory therapeutic effect in a considerable number of patients with nephrotic syndrome (NS). Interest in adrenocorticotropic hormone (ACTH) for the treatment of NS has recently been revived. The present study investigated the efficacy and safety of ACTH treatment in children with frequent relapsing NS (FRNS), steroid-dependent NS (SDNS), and steroid-resistant NS (SRNS).MethodThe ACTH treatment group was comprised of NS patients receiving ACTH treatment. Patients with serum cortisol concentrations <85.3 nmol/L and who had not received ACTH treatment previously were enrolled in the control group from January 2018 to January 2021. The maintenance dose of prednisone, the number of disease recurrences, the time of first disease relapse, immunosuppressant use, serum cortisol levels, and adverse events were recorded in both groups.ResultsFifty-one patients were included in the ACTH group, and twenty-one patients were enrolled in the control group. Concurrent treatment with one or more immunosuppressive and/or cytotoxic treatments occurred in 92.2% and 85.7% of patients in the ACTH and control groups, respectively, throughout the study period. A greater reduction in the prednisone maintenance dose was observed in the ACTH group compared with the control group after 1 year of follow up (0.603 ± 0.445 mg/kg vs. 0.267 ± 0.500 mg/kg, p = 0.006). During the one-year study period, fewer participants experienced one or more disease relapses in the ACTH group (45.1%) compared to the control group (76.2%, odds ratio = 3.896, p = 0.016). The number of disease recurrences per patient in the ACTH group was less than that in the control group (median difference = −1, p = 0.006). The mean length of remission was 8.902 m and 7.905 m in the ACTH group and control group, respectively. A log-rank test showed a longer relapse free survival for patients in the ACTH group (p = 0.046), but the Breslow test showed no significant difference between groups (p = 0.104). Ten patients in the ACTH group successfully discontinued all drug therapies. No patients in the control group were able to discontinue drug therapy as of February 2022.ConclusionACTH, combined with multiple drugs, is effective at reducing the prednisone maintenance dose and may effectively prevent disease relapses in childhood NS.

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Corticotropin and Cortisone in Management of the Nephrotic Syndrome in Children

Cited by 15 publications

References 6 publications

The Natural History of Nephrosis

The Natural History of Nephrosis

Adrenocorticotropic hormone therapy for the treatment of idiopathic nephrotic syndrome in children and young adults: a systematic review of early clinical studies with contemporary relevance

Real-word adrenocorticotropic hormone treatment for childhood-onset nephrotic syndrome

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