Decreased serum vitamin B-12 and vitamin D levels affect sleep quality in children with familial Mediterranean fever

WOS: 000419778900010PubMed ID: 29143127Familial Mediterranean fever is an autoinflammatory disorder characterized by recurrent fever and serositis. We aimed to describe serum vitamin B-12 and vitamin D levels and their correlation with self-reported sleep quality of pediatric FMF patients. Sixty-three children with FMF were enrolled to the study. Information on sleep quality was obtained using self-administration of Pittsburg Sleep Quality Index (PSQI). The patients were divided into subgroups depending on vitamin D serum concentrations: >= 20 and = 200, < 200 pg/ml. B-12 levels were not correlated with PSQI scores, whereas significant correlation was found between vitamin D and total PSQI scores and daytime sleepiness. Total PSQI score, sleep disorders and daytime sleepiness sub-scores were statistically high in patients with serum D vitamin levels below 20 ng/ml. Sleep efficiency was found lower in patients with serum B-12 levels below 200 pg/ml. B-12 may have a positive role on effective sleep. More importantly, we suggest that vitamin D is protective against sleep disorders and poor sleep, it may also improve daytime activities

MEW gene variants in children with Henoch-Schonlein purpura and association with clinical manifestations: a singie-center Mediterranean experience

WOS: 000457336800001PubMed ID: 30513227Objectives: Henoch-Schonlein purpura (HSP) is characterized by non-thrombocytopenic palpable purpura, abdominal pain, and arthralgia/arthritis. We aimed to describe the clinical presentations of children with HSP in a single center and compare the prevalence of each manifestations between patients with MEFV variants, particularly in exon 10 and those without. Methods: This cohort retrospectively included 144 HSP (59 females, 85 males) patients without Familial Mediterranean Fever (FMF) symptoms and followed for at least 6 months. We utilized the MEFV gene sequencing by using next-generation sequencing platform (MiSeq System, Illumina). Results: At least one MEFV variant was detected in 73 (50.7%) of 144 HSP patients and 5 (3.5%) patients were homozygote for M694V mutation. Although severe gastrointestinal involvement and nephritis rates were similar, we found that serum IgA, leukocyte, and platelet count at diagnosis were higher and hemoglobin was lower in HSP patients with MEFV gene variants in exon 10 than those without. Additionally, HSP patients with MEFV variants in exon 10 more often present with abdominal pain and intussusception. Conclusion: MEFV variants in exon 10 may affect clinical presentation of HSP in populations where FMF is common. While HSP may be an initial symptom of FMF, we speculate that physicians should be aware of FMF possibility in children with intussusception and lower hemoglobin, higher serum IgA, leukocyte, and platelet count.Cukurova University Scientific Research UnitCukurova University [TAY-2016-4020]This study was supported by the Cukurova University Scientific Research Unit under TAY-2016-4020

Is Henoch-Schonlein purpura a susceptibility factor for functional gastrointestinal disorders in children?

WOS: 000457425100015PubMed ID: 30101368Henoch-Schonlein purpura (HSP), the most common childhood vasculitis is characterized by non-thrombocytopenic palpable purpura, arthritis/arthralgia, abdominal pain and renal involvement. Functional gastrointestinal disorders (FGIDs) are heterogeneous disease spectrum with unclear etiology and include the most common subtypes: functional dyspepsia, irritable bowel syndrome (IBS), functional abdominal pain and functional constipation. Formerly, FGIDs were known as non-organic disorders; however, recent advances revealed that low-grade inflammation may also play a role. We aimed to clarify whether HSP predisposes to FGIDs in pediatric population. Seventy-four children with HSP, diagnosed at least 6months before the study and 78 healthy controls were enrolled to the study. Patients with red flag signs for organic GI disorders were excluded. Rome IV criteria were utilized for FGIDs diagnosis. We compared the frequencies of FGIDs between HSP patients and healthy subjects. We also examined the parameters including age, abdominal pain, arthralgia, bloody stool, renal involvement and treatment with corticosteroids and laboratory results at HSP diagnosis such as erythrocyte sedimentation rate, C-reactive protein, hemoglobin, leukocytes and platelet counts among patients with and without FGIDs. Overall FGIDs and IBS frequency were 35.1% (n = 26) and 10.8% (n = 8) in HSP patients, 19.2% (n = 15) and 2.6% (n = 2) in healthy controls, respectively. Disease characteristics and laboratory parameters at disease onset were similar between HSP patients with and without FGIDs. Overall FGIDs rate, particularly IBS were statistically higher in HSP patients. We speculate that children with preceding HSP may be predisposed to FGIDs. Since the FGIDs pathogenesis is still remains unclear, further studies are needed to confirm this hypothesis and clarify the etiology. Physicians also should pay attention to FGIDs in HSP patients with ongoing abdominal pain and thus prevent this comorbidity with dietary and psychologic measures

Clinical manifestations and outcomes of 420 children with Henoch Schonlein Purpura from a single referral center from Turkey: A three-year experience

WOS: 000496376300001PubMed ID: 31662011Objectives: Henoch Schonlein Purpura (HSP) is the most common systemic vasculitis in childhood. We aimed to evaluate the clinical features, seasonal variation, treatment outcomes and the possible predicting factors related to outcome among a large cohort of pediatric HSP patients. Methods: We conducted a medical record review study between July 2016 and January 2019 and evaluated the clinical manifestations and potential risk factors for severe gastrointestinal (GI) involvement, biopsy-proven nephritis and relapses. Results: The study included 420 HSP patients, of which the mean age at diagnosis was 7.68 +/- 3.15 years. Clinical manifestations were arthralgia and/or arthritis (n = 244, 58.1%), abdominal pain (n = 235, 56%), subcutaneous edema (n = 163, 38.8%), and renal involvement (n = 125, 29.8%). Disease recurred for at least once, in 69 (16.4%) patients and colchicine treatment yielded a favorable response in 11 of 12 relapsing patients, who did not respond to ibuprofen or steroids. Frequencies of renal involvement and biopsy-proven nephritis were higher in patients with severe GI involvement. Besides, patients with biopsy-proven nephritis had higher rates of abdominal pain, intussusception, severe GI involvement, and systemic steroid administration. Conclusion: We speculate that renal involvement, biopsy-proven nephritis and severe GI involvement can be related to each other. Colchicine may be effective in patients with relapsing disease

Recurrent Henoch Schonlein purpura without renal involvement successfully treated with methotrexate

WOS: 000471179900013PubMed ID: 30453828Introduction Henoch Schonlein purpura is characterised by palpable purpura, abdominal pain, arthritis/arthralgia, often with a self-limiting course. Herein, we report a patient with recurrent Henoch Schonlein purpura and severe gastrointestinal involvement, successfully treated with methotrexate. Case presentation A 12-year-old boy was admitted to our department with palpable purpura, abdominal pain and arthralgia. Since gastrointestinal complaints were severe, systemic steroids were administered, with tapering of dosage. Henoch Schonlein purpura recurred several times with severe abdominal pain, maelena and purpura during next two months. Colchicine and hydroxychloroquine were initiated. After four months, we also introduced methotrexate, which enabled discontinuation of previous medications including corticosteroids. Methotrexate was ceased four months later, and remission was sustained without any medications for 24 months. Conclusion Besides the conflicting data regarding the use of methotrexate in recurrent Henoch Schonlein purpura, our case introduces successful methotrexate experience in a child with Henoch Schonlein purpura and recurrent severe gastrointestinal involvement

Do practical laboratory indices predict the outcomes of children with Henoch-Schonlein purpura?

WOS: 000476946700007PubMed ID: 31021301Objectives: Henoch Schonlein Purpura (HSP) is the most common systemic vasculitis of childhood and often has a self-limiting course. We aimed to study whether practical laboratory parameters at the diagnosis predict disease course including recurrence and nephritis in addition to severe gastrointestinal involvement in children with HSP.Methods: This retrospective cohort study included 214 HSP patients, 43.5% (n = 93) female and 56.5% (n =121) male, who were diagnosed in our department. Laboratory parameters before treatment, including neutrophil, lymphocyte and platelet counts, mean platelet volume (MPV), neutrophil-to-lymphocyte (NLR), and platelet-to-lymphocyte ratios (PLR) were obtained retrospectively. Age at diagnosis, duration of follow-up, gender, preceding infections, medications, arthritis and arthralgia, abdominal pain, severe GI involvement, invagination, renal involvement and presence of nephritis, outcomes, and presence of recurrences were retrospectively recorded from medical files. Severe GI involvement was determined as severe colicky abdominal pain, bowel edema in ultrasonography or overt GI bleeding. A relapse was defined as a new flare of cutaneous lesions or other manifestations in a patient at least four asymptomatic weeks after the initial HSP episode.Results: Mean age at diagnosis was 7.6 3.1 years. Biopsy-proven nephritis was found in 16 (7.5%) patients. Severe GI involvement was present in 77 (36%) patients, whereas only 12 (5.6%) patients were diagnosed with intussusception and in 29 (13.5%) patients, HSP recurred. Neutrophil count and NLR were found higher in HSP patients with severe gastrointestinal involvement and biopsy-proven nephritis. Additionally, only platelet count was lower and MPV was higher in patients with recurrent HSP.Conclusion: Elevated neutrophil count and NLR may be relevant markers for severe GI involvement and nephritis, whereas platelet count and MPV were the only laboratory parameters associated with disease recurrence