Last update 01 Nov 2024

Pellagra Like Syndrome

Last update 01 Nov 2024

Basic Info

Synonyms

PELLAGRA-LIKE SYNDROME, Pellagra like syndrome, Pellagra-Like Syndrome

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Introduction-

100 Clinical Results associated with Pellagra Like Syndrome

100 Translational Medicine associated with Pellagra Like Syndrome

0 Patents (Medical) associated with Pellagra Like Syndrome

Literatures (Medical) associated with Pellagra Like Syndrome

01 Oct 2022·ERJ Open Research

Analysis of real-world data and a mouse model indicates that pirfenidone causes pellagra

Article

Author: Kuronuma, Koji ; Chiba, Hirofumi ; Kaneko, Shuji ; Yoshioka, Takeshi ; Yamamoto, Hiroki ; Susai, Natsumi ; Kuroita, Tomohiro

BackgroundPirfenidone (PFD) is widely used in patients with idiopathic pulmonary fibrosis (IPF) and its adverse effects, such as nausea and photosensitivity, are well known. Many patients with IPF have reduced doses or even cessation of PFD because of its side-effects. No solutions have been found for these side-effects because the current mechanistic insights are insufficient.MethodsUsing the results of real-world data analysis from the US Food and Drug Administration Adverse Events Reporting System, we hypothesised that PFD-related symptoms may be similar to pellagra. Reverse translational experiments using female BALB/c mice were performed to validate and estimate this hypothesis. Niacin and its metabolite responses were compared between patients with IPF treated with PFD and those treated without PFD.Results The pellagra hypothesis was translated from real-world data analysis. Pharmacological and comprehensive genetic investigations showed that PFD caused pellagra-related nausea and photosensitivity in a mouse model, which may have been mediated by the actions of nicotinamide N- methyltransferase (NNMT). Higher NNMT substrate responses were observed in urine from patients and mice with PFD than in those without PFD. ConclusionsPFD may cause pellagra or pellagra-like symptoms such as photosensitivity. Further studies are required to investigate whether niacin prevents pellagra-like symptoms caused by PFD in patients with IPF.

01 Feb 2019·Medecine et sante tropicales

Pellagra and pellagra-like erythema in a hospital setting in Lomé, Togo : retrospective study from 1997 to 2017.

Article

Author: Akakpo, A S ; Tchangai-Walla, K ; Moise Elegbede, Y ; Mouhari-Toure, A ; Pitche, P ; Saka, B ; Kombate, K ; Teclessou, J N

OBJECTIVEThe aim of this study was to describe the epidemiological, clinical, and therapeutic profile as well as the outcome and to document the causes of pellagra and pellagra-like erythema in a hospital setting in Lomé, Togo.METHODSThis retrospective study examined the records of patients seen for outpatient consultation and/or hospitalization in the three public dermatology departments of Lomé from January 1997 to September 2017.RESULTSDuring the study period, 178 (0.4%) of 47,219 patients seen in these dermatology departments consulted for pellagra or pellagra-like erythema; 159 (89.3%) had pellagra-like erythema. The patients' mean age was 45.8±16 years, and the sex ratio (M/F) 1.8. All patients had at least one site of cutaneous involvement, nearly always erythematous or pigmented lesions in the photo-exposed areas (99.4% of cases). Gastrointestinal and neurological signs were dominated respectively by diarrhea (12.4 % of cases), peripheral neuropathies (8.4% of cases) and insomnia (8.4% of cases). The main causes identified were alcoholism (42.1% of cases) and nutritional deficiency (6.7% of cases). Five of 178 patients were infected with HIV. All patients were treated with nicotinamide and multivitamin supplementation. No deaths were recorded during follow-up.CONCLUSIONOur results document the extreme rarity of pellagra-like erythema/pellagra. Its two main causes remain alcoholism and nutritional deficiency. Moreover, its prognosis is good when treated quickly and adequately.

01 Jan 2015·Clinical GeneticsQ2 · MEDICINE

Pellagra‐like condition is xeroderma pigmentosum/Cockayne syndrome complex and niacin confers clinical benefit

Q2 · MEDICINE

Article

Author: Alkuraya, F.S. ; Hijazi, H. ; Ibrahim, N. ; Mukhtar, M.M. ; Hamad, M.H.A. ; Hassan, H.H. ; Mohamed, K.A. ; Salih, S.B.M. ; Ansari, S. ; Salih, M.A. ; Karrar, Z.A.

An extremely rare pellagra‐like condition has been described, which was partially responsive to niacin and associated with a multisystem involvement. The condition was proposed to represent a novel autosomal recessive entity but the underlying mutation remained unknown for almost three decades. The objective of this study was to identify the causal mutation in the pellagra‐like condition and investigate the mechanism by which niacin confers clinical benefit. Autozygosity mapping and exome sequencing were used to identify the causal mutation, and comet assay on patient fibroblasts before and after niacin treatment to assess its effect on DNA damage. We identified a single disease locus that harbors a novel mutation in

ERCC5

, thus confirming that the condition is in fact xeroderma pigmentosum/Cockayne syndrome (XP/CS) complex. Importantly, we also show that the previously described dermatological response to niacin is consistent with a dramatic protective effect against ultraviolet‐induced DNA damage in patient fibroblasts conferred by niacin treatment. Our findings show the power of exome sequencing in reassigning previously described novel clinical entities, and suggest a mechanism for the dermatological response to niacin in patients with XP/CS complex. This raises interesting possibilities about the potential therapeutic use of niacin in XP.

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