Mehmet Nevzat Cizmeci

Detection of cord blood hepcidin levels as a biomarker for early-onset neonatal sepsis.

Early-onset neonatal sepsis (EONS) continues to be a severe condition associated with a high mortality and morbidity. However, symptoms and laboratory markers of this serious condition are nonspecific and currently there are no available standard tests to provide perfect diagnostic accuracy. An early recognition and initiation of antimicrobial therapy are essential in order to prevent morbidity and mortality. Hepcidin, the key regulator of iron homeostasis, is also an acute-phase reactant, which has a critical role in inflammation and contributes to host defense by interfering with microorganisms access to iron. Since hepcidin expression is induced by interleukin-6 (IL-6), it also plays role in the innate immune system. Recently, endogenous expression of hepcidin by macrophages and neutrophils in response to bacterial pathogens confirmed its role in innate immunity. The clear link between the hepcidin molecule and innate immunity may be used for the detection of EONS. We hypothesized that an increased level of hepcidin in cord blood may be used as a reliable biological marker of EONS and designed a prospective cohort study to test this hypothesis and collected pilot data. Cord blood samples of all infants born between January 2009 and December 2010 at our university hospital were collected after parental consent and a total of 38 infants were enrolled in the study who fulfilled the sepsis criteria. The range of cord blood hepcidin was found to be significantly increased in newborns with EONS (min-max: 118.1-8400 ng/mL). To the best of our knowledge, this is the first study to investigate the pathophysiologic relevance of hepcidin in EONS and demonstrate increased levels of hepcidin in cord blood as an acute-phase reactant in response to sepsis.

Cooling for Newborns with Hypoxic Ischemic Encephalopathy

adverse effects of cooling and ‘early’ indicators of neurodevelopmental outcome. Data Collection and Analysis: Four review authors independently selected, assessed the quality of and extracted data from the included studies. Study authors were contacted for further information. Meta-analyses were performed using risk ratios (RR) and risk differences (RD) for dichotomous data, and weighted mean difference for continuous data with 95% confidence intervals (CI). Main Results: We included 11 randomized controlled trials in this updated review, comprising 1,505 term and late preterm infants with moderate/severe encephalopathy and evidence of intrapartum asphyxia. Therapeutic hypothermia resulted in a statistically significant and clinically important reduction in the combined outcome of mortality or major neurodevelopmental disability to 18 months of age (typical RR 0.75 (95% CI 0.68–0.83); typical RD –0.15, 95% CI –0.20 to –0.10); number needed to treat for an additional beneficial outcome (NNTB) 7 (95% CI 5–10) (8 studies, 1,344 infants). Cooling also resulted in statistically significant reductions in mortality (typical RR 0.75 (95% CI 0.64–0.88), typical RD –0.09 (95% CI –0.13 to –0.04); NNTB 11 (95% CI 8–25) (11 studies, 1,468 infants) and in neurodevelopmental disability in survivors (typical RR 0.77 (95% CI 0.63–0.94), typical RD –0.13 (95% CI –0.19 to –0.07); NNTB 8 (95% CI 5–14) (8 studies, 917 infants). Some adverse effects of hypothermia included an increase sinus bradycardia and a significant increase in thrombocytopenia. Cochrane Abstract

Serum transaminase elevation in children with rotavirus gastroenteritis: Seven years’ experience

Abstract Background: There are no studies on clinically significant transaminase elevation due to rotavirus gastroenteritis in the literature. Also, there are significant discrepancies among previous studies regarding the prevalence of increased serum transaminase levels in rotavirus infection. Methods: Patients investigated for rotavirus by stool antigen testing, who were followed between January 2005 and May 2012, were retrospectively enrolled in this study. Patients were divided into 2 groups according to their rotavirus results: rotavirus-positive acute gastroenteritis (RPAG) and rotavirus-negative acute gastroenteritis (RNAG) groups. Results: A total of 4317 children who presented with acute gastroenteritis were assessed. The study was completed with 642 patients who met the inclusion criteria. In the RPAG group (n = 272), elevated alanine aminotransferase (ALT) was found in 42 (15.4%) patients and elevated aspartate aminotransferase (AST) in 69 (25.4%), while in the RNAG group (n = 370), these numbers were 25 (6.8%) and 44 (11.9%), respectively. The elevated ALT and AST levels were found to be significantly higher in the RPAG group than in the RNAG group (both p < 0.001). The prevalence of elevated transaminase levels was found to be similar with respect to gastroenteritis severity score (p > 0.05). The high serum transaminase levels normalized uneventfully in all patients in the RPAG and RNAG groups during follow-up. Conclusions: In this study, our results clearly signify a liver influence in rotavirus infections. Therefore, rotavirus infections should be kept in mind when evaluating the aetiology of transaminase elevation in patients with acute gastroenteritis.

Elevated level of serum osteopontin in school-age children with asthma.

BACKGROUND The role of osteopontin (OPN) has not been elucidated in childhood asthma. OBJECTIVE Our purpose was to investigate whether OPN levels change due to allergic inflammation in pre-school and school-age children. METHODS In this prospective, cross-sectional study, 42 healthy children and a total of 51 children with asthma were recruited. OPN levels and its association with clinical and laboratory parameters were investigated in the study population. The asthma group were divided into two groups with respect to age, ≤ 5-years (n = 23) and >5-years (n = 28), and labelled Asthma Group 1 and Asthma Group 2, respectively. OPN levels were compared between subgroups. RESULTS Serum OPN levels were significantly higher in the asthma group when compared to the control group (p = 0.004). OPN levels were similar in Asthma Group 1 and control groups, whereas it was found to be higher in Asthma Group 2 (p>0.025, p = 0.001, respectively). In the >5-years age asthmatic group, OPN levels of the patients with allergic rhinitis (n = 15) were higher than those of the patients (n=13) without allergic rhinitis (p = 0.021). CONCLUSION The study underscores the relationship between childhood asthma and OPN as the first study in the literature. In this study we found that OPN, which plays a role in Th2 mediated inflammation, may also play a role in childhood asthma. The fact that OPN levels do not increase in preschool-age children with asthma might be due to the transient wheezing in this group.

Is PFAPA syndrome really a sporadic disorder or is it genetic

Periodic fever syndromes are a group of disorders sharing similar symptoms, characterized primarily by regularly recurring fevers. PFAPA syndrome, one of the members of this group of disorders, is a clinical entity of unknown etiology which is frequently seen in the early childhood. Currently, the pathogenesis and the genetic basis of most of the disorders in the periodic fever spectrum are known, other than that of PFAPA syndrome. Although, classically PFAPA syndrome is known as a sporadic disease, we propose that it is not sporadic. We think that PFAPA syndrome may be an inherited disease and this hypothesis is supported by the clinical mimicry of PFAPA syndrome with other periodic fever syndromes with well-known genetic transmissions, frequent occurrence of the condition in members of the same family and emergence of common genetic mutations in the periodic fever syndrome spectrum. Moreover, our clinical observation that most of the patients diagnosed with PFAPA syndrome were of the same families strongly suggest a probable genetic transmission of this disorder. We have decided to discuss this hypothesis to contribute to the literature and assist our colleagues who are dealing with this commonly overlooked and often misdiagnosed disorder.

Decreased mean platelet volume in children with acute rotavirus gastroenteritis.

Background: The contribution of platelets to the inflammatory response via several platelet derived mediators is well recognized. The role of mean platelet volume (MPV) in infectious and inflammatory disorders, however, has not yet been well-established. While some of the previous studies demonstrated that MPV acted as a positive acute phase reactant, several others suggested its role as a negative acute phase reactant. In the current study, we aimed to assess the role of MPV as an acute phase reactant in children with rotavirus gastroenteritis. Methods: We undertook a prospective, randomized, controlled, cross-sectional study and enrolled children diagnosed with acute rotavirus gastroenteritis and healthy controls (HC), between August and November 2012. Children with acute gastroenteritis were assigned either in the rotavirus-positive acute gastroenteritis (RPAG) or in the rotavirus-negative acute gastroenteritis (RNAG) group depending on their stool antigen results. Patients were also classified into two groups based on their Vesikari score (<11: non-severe and ≥11: severe). Complete blood count and C-reactive protein (CRP) levels were assessed for all patients. We compared MPV between RPAG, RNAG and HC groups and investigated the association, if any, among MPV, platelets, white blood count and CRP. Results: In total 100 RPAG (54 males; mean age: 38.74 ± 41.45 months), 100 RNAG (58 males; mean age: 32.84 ± 29.64 months) children and 100 HC (43 males; mean age: 33.21 ± 32.55 months) were enrolled into the study. Mean platelet counts were well-matched among groups (p > 0.05). We observed a steady decline in MPV (fL) in the HC, RPAG and RNAG groups (median 7.80, 7.35 and 7.30, respectively; p < 0.0001). We did not find an association between MPV and the clinical score of gastroenteritis (p > 0.05). Conclusion: We found that MPV could be used as an acute phase reactant in children with rotavirus gastroenteritis. We believe that the current study will contribute to our understanding of MPV as an inflammatory marker.

Cerebral sinovenous thrombosis associated with MTHFR A1298C mutation in the newborn: a case report

Although cerebral sinovenous thrombosis (CSVT) is a rare condition in the neonatal period, high rates of morbidity and mortality necessitate the establishment of an early diagnosis. Methylenetetrahydrofolate reductase (MTHFR) plays a central role in the folate cycle and mutations of MTHFR are associated with vascular disease. While the C677T common missense mutation is the most well-defined MTHFR polymorphism, another common missense mutation, A1298C also exists. There has been no reported case of CSVT associated with MTHFR A1298C mutation in the neonatal period. Herein, we report a neonate with CSVT who was found to have MTHFR A1298C homozygosity.

A stitched eye in the newborn: ankyloblepharon filiforme adnatum.

Figure 2. Thin fibrous band, vertically connecting the left eyelids. A 1660 g, male infant was born at 32 weeks gestation to a 34-year-old gravida 2 para 1 mother. Pregnancy was complicated by cervical incompetence, and the infant was born via cesarean delivery. The mother denied having taken any drugs or being exposed to radiographs. There was no family history of congenital anomalies or consanguinity. On admission to the Neonatal Intensive Care Unit, the infant was noted to have a thin, threadlike band of tissue vertically connecting the left upper and lower eyelids (Figures 1 and 2). The rest of the physical examination was perfectly normal and a detailed assessment, including cranial ultrasound, echocardiography, and otoacoustic emission screening failed to reveal any other congenital anomalies. Theophthalmologic examination revealed a single adhesion in the middle portion of the left eyelid margins. With manual retraction of the eyelids, the affected eye’s anterior segment and fundus seemed normal (Figure 3). An incision to the band tissue was made with a surgical scissors with minimal hemorrhage (Video; available at www.jpeds.com). The rest of this premature infant’s course was totally normal with unremarkable detailed ophthalmologic examinations in the follow-ups. Ankyloblepharon filiforme adnatum (AFA) is defined by partial eyelid fusion with an extensile band of fibrous tissue vertically attached to upper and lower eyelids. AFA, in isolation, is of unknown etiology with an incidence of 4.4 per 100 000 births. Nonsyndromic cases might be associated

A Child with Allergic Contact Dermatitis Due to Para-Phenylenediamine

FIGURE 2. A few papular and erythematous lesions of both An 8-year-old male patient presented to our clinic with a pruritic, burning, and erythematous lesion on the left forearm 7 days after having a temporary henna tattoo done. Past medical history revealed inhaled allergen sensitivity and bronchial asthma. On physical examination, the patient had papular, erythematous, and excoriated lesions, in the shape of the initials of his and his girlfriend’s name along with a heart and arrow, on the left forearm (Figure 1). A few isolated satellite papular and erythematous lesions were also noticed at the different sites of the body (Figure 2). The patient was discharged on topical methylprednisolone and systemic antihistamine therapy. The follow-up visit 3 weeks after the initial presentation showed resolution of the papular, erythematous, and excoriated lesions with a residual hypopigmented lesion left behind (Figure 3). The standard patch test (TRUE Test; SmartPractice Denmark ApS, Hillerod, Denmark) was performed for the differential diagnosis after 6 weeks. Patch testing of the skin showed a double-positive result (erythema, infiltration, and vesiculation) for para-phenylenediamine (PPD), which clarified the final diagnosis as allergic contact dermatitis due to PPD (Figure 4). The temporary tattoos with henna are traditionally used as adornment in certain cultures, especially of Muslim and Hindu origin. Henna is obtained from the leaves of the tree Lawsonia unermis. Temporary tattoos are frequently mixed with PPD to hasten drying and to darken the color. PPD is also used in some commercial hair dyes and is a common cause of allergic reactions. Allergic reactions due to PPD in the pediatric population are on the rise in recent years and occasionally can cause severe allergic reactions; thus, the test should be performed with a 100-fold diluted standard PPD solution. Patients who have contact allergic reactions should be cautious, because hair dyes, dyes used in textile industry, and some industrial products contain PPD and may cause cross reactions. Because henna tattoos are a common practice in our country, we would like to share our

Is acute dystonia an emergency? Sometimes, it really is!

Abstract Most cases of acute dystonia are mild and easy to manage; nevertheless, some of them can be fatal because of the involvement of certain muscle groups such as the laryngeal muscles, thus requiring urgent intervention. In the literature, approach to life-threatening acute dystonia has not been investigated thoroughly, although the diagnosis is a challenge, and treatment should be offered immediately. Herein the management of life-threatening acute dystonia is discussed via 2 case reports.