Evaluation of vitamin B12 levels in pediatric familial mediterranean fever patients on colchicine treatment

GİRİŞ ve AMAÇ: Ailevi Akdeniz Ateşi (AAA), ateş ve serözit atakları ile karakterize otoinflamatuvar bir hastalıktır. Düzenli olarak kullanılan günlük 1-2 mg kolşisin tedavisi ile AAA atakları azalmakta ya da tamamen geçmektedir. Bu çalışma ile pediatrik AAA hastalarında serum vitamin B12 düzeylerinin değerlendirilmesi, kolşisin tedavisinin süresi ve dozu ile vitamin B12 düzeyi arasında ilişki olup olmadığının araştırılması planlanmıştır. YÖNTEM ve GEREÇLER: En az bir yıldır düzenli kolşisin tedavisi alan AAA tanılı 98 çocuk hasta ile yaş ve cinsiyet olarak benzer özellikteki 49 sağlıklı birey çalışmaya dahil edilmiştir. Hastalar almakta oldukları kolşisin süresine (≤ 3 yıl ve > 3 yıl) ve kolşisin dozuna (≤1 mg/gün ve > 1mg/gün) göre iki gruba ayrılmıştır. BULGULAR: Hasta ve sağlıklı bireyler arasında vitamin B12 düzeyleri arasında istatiksiksel olarak anlamlı fark saptanmadı (295,14 ± 171,62 pg/mL ile 301,55 ± 148,86 pg/mL, p=0,66). 3 yıl ve daha az süredir kolşisin tedavisi alan hastalar (310,22± 141,5 pg/ml ) ile 3 yıldan daha uzun süredir kolşisin tedavisi alan hastaların (281,81± 170,47 pg/ml) vitamin B12 düzeyleri arasında anlamlı fark saptanmadı (p=0,589). 1 mg/gün ‘den fazla miktarda kolşisin tedavisi alan hastaların medyan vitamin B12 düzeyleri, 1 mg/gün ve daha az miktarda kolşisin tedavisi alan hastaların medyan vitamin B12 düzeylerine göre anlamlı olarak düşük bulundu (197 pg/ml ile 275 pg/ml, p=0.04). TARTIŞMA ve SONUÇ: Çalışmamız ışığı altında düşünüldüğünde düzenli kolşisin tedavisi alan hastalarda vitamin B12 düzeylerinin bakılmasına gerek yoktur. Ancak yüksek dozlarda kolşisin alan hastalarda vitamin B12 düzeyinin düşük olabileceği akılda tutulmalıdır.INTRODUCTION: Familial Mediterranean fever (FMF) is an auto inflammatory disease characterized by acute, short episodes of serosal membrane inflammation and fever. Regular prophylactic treatment with colchicine at a dose of 1 – 2 mg daily prevents or substantially reduces FMF attacks and complications. The aim of this study is to evaluate the serum vitamin B12 levels in pediatric FMF patients and also compare the duration and dosage of colchicine therapy and vitamin B12 levels. METHODS: Ninety-eight pediatric FMF patients on at least one-year colchicine therapy and age and sex matched 49 healthy controls were enrolled. Complete blood count and vitamin B12 levels were measured. Patients were divided into two groups according to the duration (more than 3 years and less than 3 years) and dosage (more than 1mg/day and less than 1mg/day) of colchicine therapy. RESULTS: The mean vitamin B12 values were not significantly different between the patient and control groups (295.14 ± 171.62 pg/mL vs. 301.55 ± 148.86 pg/mL, p=0.66). Mean vitamin B12 level of the patients that were on colchicine therapy for more than 3 years (208.37±40.57 pg/ml) was not significantly different from the patients who were on colchicine therapy for less than 3 years (390.77±124.19) (p=0.0001, p<0.005). Median vitamin B12 levels were significantly lower in patients who received 1mg/day and higher colchicine therapy than the patients that received less than 1mg/day colchicine therapy (275 pg/ml vs. 197 pg/ml, p=0.04). DISCUSSION and CONCLUSION: According to our study results there is no need to evaluate serum vitamin B12 levels regularly in patients receiving colchicine treatment. On the other hand vitamin B12 levels may be decreased in patients on high dosage of colchicine

Breast-feeding-associated hypernatremia: Retrospective analysis of 169 term newborns

WOS: 000253121600006PubMed: 18279201Background: The aim of the present paper was to define the incidence, complications, morbidity and mortality of hypernatremic dehydration due to inadequate breast-feeding in a neonatal intensive care unit. Methods: A retrospective study was carried out between 2002 and 2005, to identify the term breast-fed neonates with serum sodium level >= 150 mEq/L at the Ministry of Health Ankara Diskapi Children's and Research Hospital. Results: The incidence of hypernatremic dehydration secondary to inadequate breast-feeding was 4.1%, occurring in 169 term infants among 4136 hospitalized term neonates with the following characteristics: mean gestational age, 39.1 weeks (37-42 weeks); birthweight, 3352 g (2200-4500 g); mother's age, 26.1 years (17-38 years); weight loss, 15.9% (5.4-32.7%); proportion of spontaneous vaginal deliveries, 75.7%; and proportion of first-time mothers, 74.6%. Major presenting symptoms were neonatal jaundice (47.3%) and poor infant suck (29.6%). The median sodium; blood urea nitrogen (BUN); and creatinine levels on admission were 155 mmol/L (150-194 mmol/L), 35 mg/dL (7-253 mg/dL), and 0.9 mg/dL (0.2-10 mg/dL), respectively. Major complications were as follows: acute renal failure, 82.8%; elevated liver enzymes, 20.7%; disseminated intravascular coagulation, 6.5%; brain edema, 5.2%; intracranial hemorrhage, 3.6%; cavernous sinus thrombosis, 1.2%; and bilateral iliac artery thrombosis, 0.6%. Ten patients (5.9%) developed seizure within the first 24 h of rehydration therapy with a mean sodium decrease of 11.9 mmol/L per day (4-19 mmol/L per day). Two patients (1.2%) died. There were positive correlation between weight loss and serum sodium, BUN, bilirubin levels (P 0.05). Conclusions: Hypernatremic dehydration in neonates due to inadequate breast-feeding is a serious, potentially devastating and life-threatening disorder, and can damage the central nervous system. Follow up of infants for adequate breast-feeding is important. Pediatricians must maintain a high level of suspicion, especially in cases of pathologic infant weight loss after delivery

Assessment of neutrophil to lymphocyte ratio and mean platelet volume in pediatric familial Mediterranean fever patients

Background: Blood neutrophil to lymphocyte ratio (NLR) and mean platelet volume (MPV) both have been used as a simple marker of inflammation in many disorders. Here, we aimed to investigate the relationship between NLR, MPV, and familial Mediterranean fever (FMF). Materials and Methods: In this retrospective study, the files of FMF patients in pediatric rheumatology outpatient clinic were reviewed. There were 160 participants (68.4%) in the FMF patient group and 74 participants (31.6%) in the control group. Ninety of patients were in attack-free period, and 70 were in attack period. Results: The highest values of NLR were found in the patients at attack period. Patients in attack-free period and the participants in control group had similar levels of NLR (1.71 ± 0.83 and 1.91 ± 1.86 respectively) (P = 0.457), and they had lower ratios than the patients did at attack period (4.10 ± 3.11) (P < 0.001 for both). There was no significant difference between MPV values of attack patients (8.35 ± 4.91) and attack-free patients (8.43 ± 1.15) (P = 0.074). MPV values of attack patients and attack-free patients were significantly higher than control group (7.99 ± 0.81) (P < 0.001 for both). Conclusion: NLR ratio may indicate FMF attack period. Since there was no significant difference between attack-free patients and control groups, NLR ratio cannot be used as a subclinical inflammation marker. However, NLR could be a useful predictor of inflammation in FMF patients. On the other hand, since our attack and attack-free patients have similar MPV values and both had greater MPV values than control group, we suggest that MPV may be used to show subclinical inflammation

Renal abscess in childhood: Report of five cases and review of literature

Böbrek apsesi çocukluk çağında nadir görülür ve özgün belirtileri olmadığından tanıda güçlükler olabilir. Hastaların tanısında klinik, laboratuar ve görüntüleme yöntemleri yardımcıdır. Tanıyı takiben hızla antibiyotik tedavisi başlanmalıdır. Bazı olgularda perkütan ve/veya cerrahi drenaj, ciddi olgularda ise nefrektomi gerekebilir. Bu çalışmada böbrek apsesi olan 5 hasta tartışılmış ve literatür gözden geçirilmiştir.Renal abscess is uncommon in childhood and due to nonspecific clinical features there could be diagnostic difficulties. Clinical features, laboratory findings and imaging modalities are helpful in diagnosis. Subsequent to diagnosis, antibiotic treatment should be initiated promptly. In some cases percutaneous drainage or surgery is needed. In severe cases even nephrectomy is indicated. In this study, five patients with renal abscess are discussed and literature is reviewe

The implementation of neonatal peritoneal dialysis in a clinical setting

Objective: To investigate etiology, outcome and complications related to neonatal peritoneal dialysis (PD). Methods: Neonates treated with PD in our neonatal intensive care unit during 2007-2010 were analyzed retrospectively. Results: Among 4036 hospitalized neonates; 20 neonates (0.5%) who underwent 21 cycles of PD [7 preterm, 13 term; 13 female, 7 male] were included. The mean birth weight was 2930.2 +/- 720.6 g (1120-4570), mean gestational age was 37.5 +/- 3.5 weeks (27-41). The etiologic disorders included inborn errors of metabolism (propionic acidemia, methylmalonic acidemia, citrullinemia, glutaric aciduria type2, maple syrup urine disease, 10), or acute renal failure secondary to perinatal asphyxia (4), sepsis (2), prematurity (2), hypoplastic left heart syndrome (1), kernicterus (1). The complications included peritonitis (2), early leakage (4), hemorrhage (1), catheter removal (3) and occlusion (2). The mortality rate was 50%. The gestational ages and birth weights of surviving neonates were higher (p < 0.05). Among surviving neonates, chronic renal failure (1), severe (4) and moderate neuromotor impairment (2) developed within 4-43 months. Conclusion: PD, although invasive, is an effective therapy in neonates. The complexity and invasiveness of the procedure is probably responsible for high rate of complications and mortality. If appropriate catheter selection and technique in the placement should be done, PD might improve outcome

Eculizumab therapy in a child with hemolytic uremic syndrome and CFI mutation

İstanbul Bilim Üniversitesi, Tıp Fakültesi.Background Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in childhood. It usually occurs after a prodromal episode of diarrhea and it leads to significant morbidity and mortality during the acute phase. However, cases that start as diarrhea-positive HUS whose renal function fail to recover should be screened for genetic disorders of the complement system, which is called atypical HUS (aHUS). Case-Diagnosis/Treatment We herein report a 10-year-old girl, who initially came with bloody diarrhea and had features of HUS with delayed renal and hematological recovery despite plasma therapy. Eculizumab (600 mg/week) was initiated on day 15 for atypical presentation and later a complement factor I (CFI) mutation was detected. The girl recovered diuresis within 24 h and after the third eculizumab infusion, hemoglobin, platelet, and C3 levels normalized; renal function improved; and proteinuria completely disappeared in 2 weeks. Conclusion It is our belief that eculizumab can be the treatment of choice in children who have plasma exchange-refractory HUS with defective regulation of the alternative complement pathway

Eculizumab therapy in a child with hemolytic uremic syndrome and CFI mutation

WOS: 000310829700022PubMed ID: 22903728Background Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in childhood. It usually occurs after a prodromal episode of diarrhea and it leads to significant morbidity and mortality during the acute phase. However, cases that start as diarrhea-positive HUS whose renal function fail to recover should be screened for genetic disorders of the complement system, which is called atypical HUS (aHUS). Case-Diagnosis/Treatment We herein report a 10-year-old girl, who initially came with bloody diarrhea and had features of HUS with delayed renal and hematological recovery despite plasma therapy. Eculizumab (600 mg/week) was initiated on day 15 for atypical presentation and later a complement factor I (CFI) mutation was detected. The girl recovered diuresis within 24 h and after the third eculizumab infusion, hemoglobin, platelet, and C3 levels normalized; renal function improved; and proteinuria completely disappeared in 2 weeks. Conclusion It is our belief that eculizumab can be the treatment of choice in children who have plasma exchange-refractory HUS with defective regulation of the alternative complement pathway