Clinical characteristics, quality of life and risk factors for severity in palmoplantar pustulosis: a cross-sectional, multicentre study of 263 patients.

BACKGROUND: Palmoplantar pustulosis (PPP) is a rare, chronic, inflammatory skin disease characterized by sterile pustules on palmar or plantar areas. Data on PPP are scarce. AIM: To investigate the clinical characteristics and risk factors for disease severity in a large cohort of Turkish patients with PPP. METHODS: We conducted a cross-sectional, multicentre study of patients with PPP recruited from 21 tertiary centres across Turkey. RESULTS: In total, 263 patients (165 women, 98 men) were evaluated. Most patients (75.6%) were former or current smokers. The mean Palmoplantar Pustulosis Area and Severity Index (PPPASI) was 8.70 ± 8.06 and the mean Dermatology Life Quality Index (DLQI) score was 6.87 ± 6.08, and these scores were significantly correlated (r = 0.52, P < 0.001). Regression analysis showed that current smoking was significantly associated with increased PPPASI (P = 0.03). Coexisting psoriasis vulgaris (PsV) was reported by 70 (26.6%) patients. Male sex prevalence, PPP onset incidence, disease duration, DLQI, and prevalence of nail involvement and psoriatic arthritis (PsA) were significantly increased among patients with PPP with PsV. Of the 263 patients, 18 (6.8%) had paradoxical PPP induced by biologic therapy, and these patients had significantly increased mean DLQI and prevalence of PsA (r = 0.03, P = 0.001). CONCLUSION: Our data suggest that smoking is a risk factor for both PPP development and disease severity. Patients with PPP with PsV present distinct clinical features and patients with biologic therapy-induced paradoxical PPP have reduced quality of life and are more likely to have PsA.

Mean platelet volume/count and mortality in extremely low birth weight infants of preeclamptic mothers.

PURPOSE: To compare complete blood count (CBC) parameters in extremely low birth weight (ELBW) infants born to mothers with and without preeclampsia and to evaluate whether these parameters could be used to determine the prognosis of infants born to mothers with preeclampsia. METHOD: Thirty-eight infants of preeclamptic mothers (IPM) and 77 infants of non-preeclamptic mothers (INPM) were included in the study. The CBC parameters of ELBW infants were evaluated at the sixth hour of life. RESULTS: The mean hemoglobin level of the IPM group was higher than the INPM group (16.4±2.4 vs 15.3±2.4; p=0.02). The mean platelet count of the IPM Group was significantly lower than the INPM group (168±65 vs 206±78; p=0.008). Overall and 7th day of life survival of infants were not different between the groups, but there was a correlation between platelet count of the IPM group and mortality in the first 7 days of life and overall mortality (r=-0.38, p=0.023 and r=-0.36, p=0.029). A cut-off point of 0.4 had significant predictive value for mortality (sensitivity of 91%, specificity of 66%). CONCLUSION: Hemoglobin and platelet counts were statistically different in ELBW infants born to preeclamptic mothers compared with non-preeclamptic mothers. Although the survival was not different between the two groups, platelet count and MPV/platelet count ratio were significantly correlated with overall mortality and mortality in the first 7 days of life in infants of preeclamptic mothers.

Effect of maternal cervical bacterial colonization on neonatal outcome in high-risk pregnancies: results from a tertiary maternity center in Turkey.

PURPOSE: To evaluate and compare the morbidity and mortality of neonates born to pregnant women with positive and negative cervical cultures. MATERIALS AND METHODS: The demographic and clinical features of mothers included in this study, along with details of the microorganisms isolated on maternal cervical cultures and the number of days between a positive cervical culture and delivery were recorded. Neonates were stratified into two groups based on cervical culture results of their mothers--Group 1, positive cervical culture; Group 2, negative cervical culture. RESULTS: A total of 216 women who delivered 242 infants were included in the study. Group 1 consisted of 90 neonates while Group 2 had 152 newborns. The difference between the groups with demographic characteristics was statistically insignificant. Mean levels of the acute phase reactants, CRP, and IL-6, obtained six hours after delivery were significantly higher in Group 1 compared to Group 2 (p < 0.05 for C-reactive protein (CRP) andp < 0.001 for IL-6). Although there was no difference between groups in terms of duration of respiratory support, mean duration of hospitalization, as well as mortality rate were significantly higher in Group 1 (p < 0.001, p < 0.05, respectively). CONCLUSIONS: Women diagnosed with a high-risk pregnancy should be treated with antibiotics immediately after a positive cervical culture result, and delivery should be delayed until the success of antibiotic treatment can be evaluated. Early initiation of maternal antibiotic therapy is associated with shorter durations of hospital stay for newborns. Close follow-up of mothers with high-risk pregnancies and extension of treatment duration are critical for determining prognosis in newborn infants.

Fryns anophthalmia-plus syndrome: two rare cases.

Fryns anophthalmia-plus syndrome is a rare syndrome with clinical diversity primarily including anophthalmia/microphthalmia, facial clefts, cleft lip/palate, ear and nasal deformities. Here we present two different cases of APS with anopthalmia/microphthalmia, cleft palate, low set ears, ventriculomegaly and one of which had intestinal non-fixation anomaly not described in the literature before.

Infliximab-induced discoid lupus erythematosus.

Anti-tumor necrosis factor-alpha (TNF-α) immunotherapy is currently used in the treatment of various inflammatory diseases such as rheumatoid arthritis, psoriasis, psoriatic arthritis, ankylosing spondylitis and Crohn's disease. Infliximab is a chimeric monoclonal antibody that binds to transmembrane-bound and soluble TNF-α. Previously, a discoid lupus erythematosus-like eruption linked to its use was rarely reported in patients with rheumatoid arthritis. We present a case of rheumatoid arthritis which developed such an eruption after treatment with infliximab. The lesions resolved after the discontinuation of infliximab. In the present case, the clinical, serological and immunohistochemical features of our patient are discussed with the literature.

Mean platelet volume in very preterm infants: a predictor of morbidities?

BACKGROUND: Mean platelet volume [MPV] is an important predictor for many diseases and larger platelets are more reactive and associated with shortened bleeding time. Although elevated MPV values are related to respiratory distress syndrome [RDS] in neonates, there are, to our knowledge, no data investigating the relationship between MPV and other diseases of preterm infants. AIM: To assess the correlation between MPV and the occurrence of various morbidities of prematurity such as necrotizing enterocolitis [NEC], bronchopulmonary dysplasia [BPD], sepsis, retinopathy of prematurity [ROP], and intraventricular hemorrhage [IVH] in a cohort of very preterm infants. SUBJECTS: We studied infants with a gestational age of < 34 weeks and a birth weight of < 1500 g admitted to a third level Neonatal Intensive Care Unit. Enrolled infants were divided into NEC and non-NEC, sepsis and non-sepsis, ROP and non-ROP, BPD and non-BPD and IVH and non-IVH groups. MPV was evaluated at birth [cord blood] and repeated at 48-72 hours of life. RESULTS: Two hundred and seventy two infants were studied. MPV measured at birth was similar between sepsis and non-sepsis, and ROP and non-ROP groups. MPV values were higher in infants with BPD [9.08±1.3 fl], IVH [8.4±1.1fl] and NEC [8.6±0.7 f] when compared to the control group [7.6±0.6 fl] in the first day of life. CONCLUSIONS: High MPV in the first hours of life may reflect the presence of a risk factor for the development of NEC, BPD and IVH in extremely preterm infants. This might be associated with inflammatory and oxidative process. However, our data indicate that higher MPV values are not associated with the development of sepsis or ROP in this study population.        

Adiponectin and visfatin levels in extremely low birth weight infants; they are also at risk for insulin resistance.

AIM: The aim of this study was to assess adiponectin, visfatin, HOMA-IR, glucose and triglyceride levels in term, preterm and extremely low birth weight (ELBW) babies. Each of these three groups was subdivided into two groups as small-for-gestational age (SGA), and appropriate-for-gestational age (AGA). 30 term, 30 preterm and 30 extremely low birth weight infants were included into the study. RESULTS: There was no significant difference in term and preterm infants for serum adiponectin, visfatin, and HOMA-IR levels. There were also no significant differences between term and preterm infants for glucose and triglycerides. The serum visfatin, insulin and HOMA-IR levels (p = 0.001, p = 0.001 and p < 0.05, respectively) were higher in ELBW group than preterm group. Comparing the subgroups as SGA and AGA in all main groups, only in ELBW group there were no significant differences in serum adiponectin, visfatin, HOMA-IR and insulin levels. CONCLUSIONS: We suggest that visfatin can be used as an early indicator of insulin resistance. Independent of being SGA, ELBW itself may be a risk factor for insulin resistance. In the follow-up of these babies the risk of obesity, metabolic syndrome and cardiovascular diseases may be increased as in SGA babies.  

An infant with congenital fibula deficiency accompanying with deafness.

Congenital longitudinal deficiency of the fibula (CLDF) is the most common congenital defect involving the long bones. There have been many different classifications developed for fibula deficiency. Achterman and Kalamchi's classification is most commonly used and will be described. Our case was complied with type II. Other anomalies includes cardiac anomalies, thrombocytopenia absent-radius (TAR) syndrome, thoracoabdominal schisis, spina bifida and renal anomalies, but most associated anomalies are skeletal. We here presented firstly in literature an infant with congenital fibula deficiency accompanying with deafness.

Hydrocortisone acetate alone or combined with mupirocin for atopic dermatitis in infants under two years of age - a randomized double blind pilot trial.

BACKGROUND: The skin of patients with atopic dermatitis (AD) is heavily colonized with Staphylococcus (S.) aureus, even at uninvolved sites. Toxins secreted by the majority of S. aureus on the skin behave as superantigens and can directly influence the disease activity, although clinical signs of bacterial superinfection might be absent. OBJECTIVES: This study was conducted to compare the efficacy of hydrocortisone cream, combined with mupirocin or alone with emmolient ointment for the treatment of mild to moderate AD in infants between six months and two years of age. MATERIALS AND METHODS: A total of 83 patients with mild to moderate AD were randomized to receive hydrocortisone, hydrocortisone+ mupirocin or emmolient ointment twice daily in one week and followed-up for 8 weeks, in a blind study. Efficacy evaluation made by SCORAD and eczema area and severity index (EASI) at baseline, day 7, and weeks 2, 4, and 8. Possible adverse events were recorded to evaluate safety. RESULTS: At the end of study, 65% (17 of 26) of the patients were treated successfully with hydrocortisone ointment based on SCORAD and EASI scores. Also there was a significant improvement in patients combined with mupirocin ointment [74% (20 of 27)]. The percent improvement from baseline in EASI scores was also significantly greater in hydrocortisone and combined group compared with emmolient-treated patients (36%) (p = 0.0187, p = 0.012 respectively). CONCLUSIONS: Monotherapy with hydrocortisone ointment is the main treatment in infants with mild to moderate AD and combination with mupirocin is safe and effective often needed because of possible Staphylococcus carriage.

Severe semilobar holoprosencephaly and lissencephaly associated with cebocephaly in a newborn.

Holoprosencephaly is frequently accompanied by midline facial abnormalities such as hypotelorism, cyclopia, etmocephaly and cebocephaly. Cebocephaly is a very rare congenital anomaly combining with semilobar holoprosencephaly. Chromosomal analysis shows normal karyotyping. Lissencephaly and holoprosencephaly are rare associations, that have not been reported yet with cebocephaly. Herein we present the first case of cebocephaly with severe semilobar holoprosencephaly and lissencephaly.

Can first trimester pregnancy-associated plasma protein-A predict the surfactant needs of preterm neonates?

BACKGROUND: Low values of pregnancy associated plasma protein A (PAPP-A), have been shown to be associated with some poor gestational outcomes, especially those related to placental deficiency such as pre-eclampsia and fetal growth restriction as well as preterm labor. The aim of this study was to compare first trimester PAPP-A MoM values with the surfactant needs of newborns of pregnant women who had a preterm delivery. METHODS: This study included 216 pregnant women who had a preterm delivery, who were found to be in the low-risk group based on their aneuploidy screening. The women were separated into two groups based on the surfactant receipts of their newborns. A record was made of the obstetric history, birth characteristics of the preterm infants, and whether or not there was a need for surfactant. RESULTS: A comparison of the PAPP-A values of the two groups revealed that the group that received surfactant had statistically significantly lower PAPP-A values (t(-3.97) = 0.203, p < 0.001). When the cut-off value of PAPP-A was taken as 1 MoM and the gestational age was analyzed together with the birth weight, PAPP-A alone was found to be a significant independent variable for the prediction of respiratory distress syndrome (RDS) (p = 0.031; OR:8.2 (1.2-55.6)). CONCLUSIONS: The result of this study demonstrated that PAPP-A MoM values may be significant in predicting the need for surfactant in RDS, which is a frequently seen condition in the neonatal period.

Factors associated with neurodevelopmental impairment in preterm infants with bronchopulmonary dysplasia.

OBJECTIVE: Bronchopulmonary dysplasia (BPD) is a common and serious complication in preterm infants with very low birth weight and is known to lead to poor neurodevelopmental outcomes. This study aimed to identify factors associated with neurodevelopmental impairment (NDI) in patients with moderate to severe BPD. SUBJECTS AND METHODS: A total of 83 preterm infants born between 24- and 29-weeks' gestation who were admitted to the neonatal intensive care unit and developed moderate/severe BPD between 2013 and 2017 were retrospectively evaluated. Developmental assessment was performed at 18 to 24 months of corrected age using the Bayley Scales of Infant Development II (BSID-II). Patients with NDI (n=41) and without NDI (n=42) were compared. RESULTS: BSID-II Mental Development Index and Psychomotor Development Index scores were 87±11 and 83±8 in the non-NDI group and 57±12 and 52±8 in the NDI group, respectively (p<0.001). The NDI group had significantly lower birth weight (847±174 vs. 1012±192 g) and gestational age (26.1±1.3 and 27.6±1.6 weeks) compared to the non-NDI group (p<0.001). Intraventricular hemorrhage, periventricular leukomalacia, retinopathy of prematurity, exposure to steroids, duration of respiratory support, and length of hospital stay were significantly higher in the NDI group (p<0.001). CONCLUSIONS: Many of the conditions in this study were found to be associated with poor neurodevelopmental outcomes in patients with BPD, such as prolonged respiratory support, prolonged hospitalization, intraventricular hemorrhage, retinopathy, and steroid therapy, can be avoided or prevented with strict protocols and prevention strategies. Appropriate management of comorbid risk factors may help prevent poor neurodevelopmental outcomes.

Effect of withholding feeds on transfusion-related acute gut injury in preterm infants: a pilot randomized controlled trial.

Background: Several retrospective studies have reported an increase in necrotizing enterocolitis (NEC) during the 48 h following red blood cell (RBC) transfusion. Whether withholding enteral feeding during transfusion decreases the risk of transfusion-associated acute gut injury (TRAGI) in preterm infants is unclear.Study design and methods: In this pilot study, 112 preterm infants with gestational age ≤32 weeks and/or birth weight ≤1500 g were randomly assigned to withholding (NPO) or continuance of feeding (FED) during RBC transfusion. Primary outcome measure was development of NEC (stage ≥ 2) within 72 h of a transfusion and the change in abdominal circumference.Results: One hundred fifty-four transfusion episodes (74 NPO and 80 FED) were analyzed. Demographic characteristics were found to be similar in both groups. There was no difference in rates of NEC (0 versus 3.4%; p = .49) between the NPO and FED groups. The incidence of feeding intolerance was higher in the FED group; however, it was statistically insignificant (1.9 versus 6.8%, p = .36). Abdominal circumference remained similar in both groups in all three consecutive days following transfusion (p>.05).Conclusion: This pilot study does not support withholding feedings during transfusion but is not adequately powered to test the hypothesis that NPO decreases NEC rates. Adequately powered well-designed multicenter trials are still required.

Does individual room implemented family-centered care contribute to mother-infant interaction in preterm deliveries necessitating neonatal intensive care unit hospitalization?

The aim of the study was to investigate the effect of individual room care in the neonatal intensive care unit (NICU) on the factors that influence mother-preterm infant interaction. Mothers in group I had hospitalization with their preterm infants in an individual room in the NICU. Mothers in group II were not hospitalized but had opportunity to visit their babies and spend time with them whenever they wanted. On the postdischarge third month, mothers were assessed for parental stress, postpartum depression, and perception of vulnerability. Although the mean depression, stress, and vulnerability scores were higher in group II, there was no significant difference between the groups (P > 0.05). Postpartum depression rate was more than double in group II, but this difference was not statistically significant (P = 0.06). Individual room care in the NICU cannot prevent maternal stress, postpartum depression, and perception of vulnerability related to having a high-risk preterm infant by itself alone.

Preeclampsia is an independent risk factor for spontaneous intestinal perforation in very preterm infants.

BACKGROUND: Spontaneous intestinal perforation (SIP) is an important surgical emergency in preterm infants. AIMS: To evaluate the effect of maternal preeclampsia on development of SIP in premature infants. STUDY DESIGN: Retrospective observational study in a large tertiary neonatal intensive care unit. SUBJECTS: The preterm infants of ≤32 weeks of gestational age and birthweight ≤1500 g who were hospitalized were enrolled. OUTCOME MEASURES: The primary outcome was to determine the association between preeclampsia and SIP. RESULTS: A total of 22 infants had SIP diagnosis. The incidence of SIP in infants born to preeclamptic mothers (6.2%) was significantly higher compared with those born to normotensive mothers (0.2%). In multinominal logistic regression model, preeclampsia was found to be an independent risk factor of SIP with an odds ratio of 13.5 (95% confidence interval 2.82-65.1). CONCLUSIONS: Maternal preeclampsia seemed to be an independent risk factor for development of SIP in premature infants.

Surfactant administration via thin catheter during spontaneous breathing: randomized controlled trial.

BACKGROUND: The primary aim of this randomized study was to describe the feasibility of early administration of surfactant via a thin catheter during spontaneous breathing (Take Care) and compare early mechanical ventilation (MV) requirement with the InSurE (Intubate, Surfactant, Extubate) procedure. METHODS: Preterm infants, who were <32 weeks and stabilized with nasal continuous positive airway pressure (nCPAP) in the delivery room, were randomized to receive early surfactant treatment either by the Take Care or InSurE technique. Tracheal instillation of 100 mg/kg poractant α via 5-F catheter during spontaneous breathing under nCPAP was performed in the intervention group. In the InSurE procedure, infants were intubated, received positive pressure ventilation for 30 seconds after surfactant instillation, and placed on nCPAP immediately. RESULTS: One hundred infants in each group were analyzed. The MV requirement in the first 72 hours of life was significantly lower in the Take Care group when compared with the InSurE group (30% vs 45%, P = .02, odds ratio -0.52, 95% confidence interval -0.94 to -0.29). Mean duration of both nCPAP and MV were significantly shorter in the Take Care group (P values .006 and .002, respectively). Bronchopulmonary dysplasia rate was significantly lower among the infants treated with the Take Care technique (relative risk -0.27, 95% confidence interval -0.1 to -0.72) CONCLUSIONS: The Take Care technique is feasible for the treatment of respiratory distress syndrome in infants with very low birth weight. It significantly reduces both the need and duration of MV, and thus the bronchopulmonary dysplasia rate in preterm infants.

The association between respiratory tract Ureaplasma urealyticum colonization and severe retinopathy of prematurity in preterm infants ≤1250 g.

AIM: To evaluate the association between respiratory tract Ureaplasma urealyticum (Uu) colonization and development of retinopathy of prematurity (ROP) requiring treatment. METHODS: The infants with birthweight (BW) ≤1250 g born in a third-level neonatal intensive care unit between March 2009 and May 2010 were prospectively identified. Nasopharyngeal swabs for Uu colonization were taken in postnatal first 3 days. Culture-positive patients were reevaluated on the twelfth day by nasopharyngeal swabs for Uu. The primary outcome was to define whether there was an association between respiratory tract Uu colonization and severe ROP requiring treatment. Independent sample's t-test or Mann-Whitney U-test was used to compare continuous variables and Chi-square test or Fisher's exact test for categorical variables. Multivariate (backward) logistic regression analysis was performed to simultaneously measure the influence of the independent variables with ROP as the dependent variable. RESULTS: A total of 25 (12.1%) infants developed severe ROP requiring treatment among 206 infants who underwent ROP screening. Mean BW and gestational age of total cohort were 1013±159 g and 27.9±1.6 weeks, respectively. Multivariate analysis demonstrated that BW (OR: 0.64 (95% Cl 0.47-0.88); P=0.006), duration of mechanical ventilation (OR: 1.17 (95% Cl 1.06-1.28); P=0.001), premature rupture of membrane >18 h (OR: 3.83 (95% Cl 1.2-12.2); P=0.02), and Uu positivity in both cultures (OR: 5.02 (95% Cl 1.8-13.9); P=0.002) were independent risk factors for the development of severe ROP requiring treatment. CONCLUSIONS: Respiratory tract colonization with Uu was independently associated with severe ROP requiring treatment.