Should children with psoriasis be consulted to a rheumatologist? Result from pediatric rheumatology-dermatology collaboration.

OBJECTIVE: The objectives of this study were to determine the musculoskeletal (MSK) conditions associated with pediatric psoriasis (Pso) and to evaluate the thickness of Achilles tendon of children with Pso and healthy controls (HCs). METHODS: Pso patients who were followed-up in dermatology outpatient clinic were referred to a pediatric rheumatology center. All patients and healthy peers were evaluated with standardized forms. Both patients and controls underwent ultrasonographic evaluation for Achilles tendon thickness. RESULTS: A total of 55 pediatric Pso and 46 healthy children were included in the study. Of patients with Pso 56.4% had arthralgia, 25.5% had lower back pain, 18.2% had heel pain, 12.7% had hip pain, and 10.9% described morning stiffness. Arthritis was detected in 7.3%, sacroiliac tenderness in 12.7%, and enthesitis in 9.1% of the patients. Arthralgia, lower back pain, and heel pain were significantly frequent in Pso group than healthy children median left and right Achilles tendon thicknesses of Pso patients who were significantly greater than that of HCs prevalence of psoriatic arthritis (PsA) among Pso patients was 7.3%. CONCLUSION: Evaluation of a child with Pso regularly for the MSK complaints is critical for the early recognition of PsA. Ultrasonography is a useful technique for screening Pso patients for early detection of enthesopaty.

Sacroiliitis in children and adolescents with familial Mediterranean fever.

BACKGROUND: Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by recurrent episodes of fever and serositis. Sacroiliitis can be observed in some FMF patients. This study aimed to compare the demographic, clinical, and laboratory findings, and treatment in children with FMF and sacroiliitis, and children with juvenile spondyloarthropathy (JSpA). METHODS: In total, 1687 pediatric FMF patients that were followed-up between May 2010 and June 2020 were evaluated retrospectively. Among them, those with sacroiliitis (n = 63) were included in the study and compared to patients with JSpA (n = 102). RESULTS: The study included 63 FMF patients with sacroiliitis (38 males [60.3%] and 25 females [39.7%]) with a mean age of 15.2 ± 4.1 years. Mean age at symptom onset was 7.2 ± 5.05 years and mean age at diagnosis was 9.74 ± 4.67 years. The most common mutation in the FMF patients was M694V/M694V (n = 22). Patients were diagnosed with sacroiliitis with a mean of 12 months (range: 6-36 months) after the diagnosis of FMF. Among the FMF patients, 28 (44.4%) had enthesitis, 23 (36.5%) had heel pain, and 11 (17.4%) had low back pain. The study also included 102 JSpA patients (90 males [88.2%] and 12 females [11.8%]). Mean age of patients with JSpA was 16.1 ± 2.8 years. As compared to 102 JSpA patients, patients with FMF and sacroiliitis had higher acute phase reactants, whereas HLA-B27 positivity rate was lower. In addition, axial involvement rate was higher in the JSpA patients. CONCLUSION: Sacroiliitis is a common co-morbidity in FMF patients. The phenotypic features of these patients are different from patients with JSpA.

Follow-Up of Low Birth Weight Preterm Infants After Hospital Discharge: Incidence and Reasons for Rehospitalization.

OBJECTIVES: The rehospitalization frequency/indications of low birth weight (LBW) preterms and the effect of rehospitalization on growth and neurodevelopment were investigated. METHODS: LBW preterms discharged from NICU were prospectively followed until the corrected age of 1 year. Infants rehospitalized after discharge were defined as the study group and those not rehospitalized as the control group. The frequency, duration and etiology of rehospitalization were investigated and the effects of neonatal complications, surgery and socio-demographic status on rehospitalization were assessed. RESULTS: The study and the control group included 113 and 217 infants, respectively. Infants in the study group were rehospitalized 247 times in total. Rehospitalization was significantly higher in the male gender (39.7% vs. 28.9%, p < 0.05). Hyperbilirubinemia-anemia, anemia-surgery and pulmonary-other infections were the most common indications for rehospitalization in the 0-14 days, 15 days to 2 months and 2-12 months, respectively. Intrauterine growth had no impact on rehospitalization. Somatic growth and neurodevelopment were significantly delayed in the study group (p < 0.05). CONCLUSION: Birth weight and gestational week are the most important determinants of rehospitalization. Rehospitalized preterm infants have significant deficits in both somatic growth and neurodevelopment despite high-quality follow-up care.

Sacroiliitis in children and adolescents with familial Mediterranean fever

Abstract Background: Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by recurrent episodes of fever and serositis. Sacroiliitis can be observed in some FMF patients. This study aimed to compare the demographic, clinical, and laboratory findings, and treatment in children with FMF and sacroiliitis, and children with juvenile spondyloarthropathy (JSpA). Methods: In total, 1687 pediatric FMF patients that were followed-up between May 2010 and June 2020 were evaluated retrospectively. Among them, those with sacroiliitis ( n = 63) were included in the study and compared to patients with JSpA ( n = 102). Results: The study included 63 FMF patients with sacroiliitis (38 males [60.3%] and 25 females [39.7%]) with a mean age of 15.2 ± 4.1 years. Mean age at symptom onset was 7.2 ± 5.05 years and mean age at diagnosis was 9.74 ± 4.67 years. The most common mutation in the FMF patients was M694V/M694V ( n = 22). Patients were diagnosed with sacroiliitis with a mean of 12 months (range: 6-36 months) after the diagnosis of FMF. Among the FMF patients, 28 (44.4%) had enthesitis, 23 (36.5%) had heel pain, and 11 (17.4%) had low back pain. The study also included 102 JSpA patients (90 males [88.2%] and 12 females [11.8%]). Mean age of patients with JSpA was 16.1 ± 2.8 years. As compared to 102 JSpA patients, patients with FMF and sacroiliitis had higher acute phase reactants, whereas HLA- B27 positivity rate was lower. In addition, axial involvement rate was higher in the JSpA patients. Conclusion: Sacroiliitis is a common co-morbidity in FMF patients. The phenotypic features of these patients are different from patients with JSpA.

Assesment of perinatal mortality in two different periods: results of a single center.

AIM: This study aimed to investigate the perinatal mortality rate with 37 864 deliveries which occured in two different periods in a single center, to compare the components of perinatal mortality and affecting factors with the results of the study related with perinatal mortality which we conducted in 1999 and to emphasize the precautions directed to reduce mortality rates. MATERIAL AND METHODS: All live births and stillbirths which occurred in Bakirköy Obstetrics and Pediatrics Training and Research Hospital between January 2007 and December 2007 were evaluated. The results were compared with the results of the study conducted in 1999. Newborns with a weight above five hundred grams and a gestational age above 22 weeks were enrolled in the study. The stillbirth rate, early neonatal mortality rate, late neonatal mortality rate, perinatal mortality rate and corrected perinatal mortality rate were calculated. Modified Wigglesworth Classification was used for evaluating the perinatal mortality and the subjects were examined in 7 groups. The characteristics belonging to the years of 2007 and 1999 were examined, the differences were recorded and the results were discussed. When the two periods were compared, it was observed that the perinatal mortality rate increased from 23.5‰ to 26‰. RESULT: When the causes were investigated, it was observed that the stillbirth rate was increased in 2007 (84%) and especially congenital anomalies had an important role in this increment. The early neonatal mortality rate declined from 0.8% in 1999 to 0.4% in 2007. It was found that especially the premature mortality rate (Group 3) and the mortality rate related with perinatal asphyxia (Group 4) were significantly decreased. CONCLUSION: The decrease in early neonatal mortality rate could be best explained by productive operation of the new neonatal intensive care unit which had been established after 2002.

Neurodevelopment of preterm infants born after in vitro fertilization and spontaneous multiple pregnancy.

BACKGROUND: The aim of this study was to compare perinatal, neonatal characteristics and neurodevelopmental prognosis of preterm infants born after in vitro fertilization (IVF) and spontaneous multiple pregnancy, and to evaluate the factors affecting neurodevelopmental outcome at 24-36 months. METHODS: A total of 125 preterm infants, 65 from spontaneous and 60 from IVF multiple pregnancy were evaluated in terms of neurodevelopmental outcome at the age of 24-36 months. Mean maternal age, chronic maternal disease, birthweight, gestational week, gender, APGAR score, neonatal intensive care unit admission, presence of congenital anomalies, referral to follow up, rehospitalization and socioeconomic status were investigated. Gross Motor Function Classification System and Denver II Developmental Screening Test were carried out. Local ethics committee approved the study (12.10.2010; no: 305). RESULTS: Mean maternal age, chronic maternal illness, pregnancy-related diseases, 5 min APGAR score, rate of cesarean delivery and referral to follow up were significantly higher in the IVF group (P < 0.05). Neurological examination identified increased muscle tone in two children (1.6%); only one infant in the IVF group had cerebral palsy. A total of 26 subjects (20.8%; spontaneous group, n =17, 26.2%; IVF group, n = 9, 15%) had abnormal Denver II findings, mostly in language (8.8%) and personal-social (8.0%) development. CONCLUSION: Morbidity, length of hospitalization and neurodevelopmental outcome of preterm infants born after spontaneous and IVF multiple pregnancy are similar. Delays in language and personal-social development were the most common neurodevelopmental abnormalities. Even within similar socioeconomic status, parents in the IVF group were more compliant with follow up.

School Performance and Neurodevelopment of Very Low Birth Weight Preterm Infants: First Report From Turkey.

Very low birth weight preterm infants are under significant risk of neurologic, developmental, and somatic problems. In this study, 90 infants born with a birth weight <1500 g and/or with a gestational age <32 weeks were evaluated after the first year of elementary school to assess neurodevelopment. The Wechsler Intelligence Scale for Children-Revised (WISC-R) test, Pediatric Symptom Checklist, and Parent Evaluation of Developmental Status were performed. Mental retardation, cerebral palsy, blindness, epilepsy, and posthemorrhagic hydrocephaly incidences were 14%, 7%, 2%, 5%, and 2%, respectively. The WISC-R score of 32 patients (35.5%) were below 85. Perinatal asphyxia, abnormal neurologic examination, and delayed or impaired speech correlated significantly with low WISC-R scores. Education and income of the father had positive impact on WISC-R scores (P = .042 and P = .026). Parents' concern and presence of cognitive problems were correlated (P = .026). Environmental factors, as well as the prevention of morbidity, affected school performance positively.

Systemic onset juvenile idiopathic arthritis with macrophage activation syndrome and coronary artery dilatation misdiagnosed as Kawasaki disease.

Systemic onset juvenile idiopathic arthritis (SoJIA) is characterized by arthritis, fever and visceral organ involvement including hepatosplenomegaly, lympadenopathy and serositis. This is a case of SoJIA misdiagnosed as Kawasaki disease (KD) and developed machrophage activation syndrome (MAS) secondary to Ebstein-Barr virus (EBV) infection. It is presented to point out the conditions that may come along. First of all, SoJIA should be kept in mind while making the differential diagnosis of coronary arterial ectasias and dilatations usually seen in vasculitic diseases like KD. Second, as a very fatal complication MAS should always be considered while following a patient with the diagnosis of SoJIA. Infections like EBV may be the potential triggers for development of MAS especially in immunesupressed patients.

Familial mediterranean Fever: diagnosing as early as 3 months of age.

Familial Mediterranean Fever is an autosomal recessive disease. Major symptoms of disease are recurrent fever accompanied by serositis attacks. The disease is usually diagnosed before 20 years of age. Symptoms related to FMF are noted when children become more verbal, usually after 2 years of age. In this case report, the youngest patient with the diagnosis of FMF is presented. She was consulted to pediatric rheumatology for the high acute phase response and fever. It was learned that her mother had recurrent swelling of her ankle joints. Mutation analysis was performed and two homozygous mutations (M694V and R202Q) were identified. She was diagnosed as FMF at 3 months of age and colchicine was started. She responded to colchicine. Her uncontrolled acute phase response declined gradually. This case was reported to point out the importance of early remembrance of autoinflammatory diseases even at very early ages especially at endemic countries.

Features of multiple pregnancies obtained by in vitro fertilization or spontaneously.

BACKGROUND: The aim of this study was to investigate the prognosis of infants born to multiple pregnancies obtained by in vitro fertilization (IVF) or spontaneously. METHODS: The records of multiple pregnancies in Bakirkoy Maternity and Children's Hospital between February 2008 and January 2009 were investigated. Maternal, perinatal and postnatal data of multiple pregnancies were evaluated. The congenital anomalies determined by physical examination and/or imaging studies in the prenatal, natal and postnatal period and the difference between groups in terms of morbidity and mortality were investigated as well. RESULTS: A total of 401 spontaneous multiple pregnancies and 128 IVF multiple pregnancies were included in the study. Rate of multiple pregnancies for live births and IVF rate were 3.8% and 0.79%, respectively. The mean maternal age was 30.1 years (21-43) in the IVF group and 27.9 years (13-43) in the spontaneous group (P < 0.05). The cesarean delivery rate was 100% in the IVF group and 78% in the spontaneous group (P = 0.002). Premature rupture of membranes rate was 9.8% in the IVF group and 3.6% in the spontaneous group (P < 0.05). CONCLUSIONS: Multiple pregnancies constitute an important health problem due to high perinatal risks and increased health costs. We found maternal age, premature rupture of membranes and cesarean delivery rate significantly high in the IVF group. No significant differences were found between spontaneous and IVF multiple pregnancies in terms of demographic features, hospitalization stay and rate, admission to neonatal intensive care unit, mortality and congenital malformation.

Perinatal and somatic growth properties of preterm babies born from spontaneous and in vitro fertilization multiple pregnancies.

AIM: The objective of this study was to examine perinatal and neonatal properties of preterm infants with a corrected age of 24-36 months who were born as a result of spontaneous and in vitro fertilization multiple pregnancies, to interrogate somatic growth properties of these infants and evaluate the factors which had an impact by comparing groups. MATERIAL AND METHODS: A total of 125 children with a birth weight below 2 500 g and a gestational age below the 37(th) gestational week 60 of whom were born as a result of in vitro fertilization multiple pregnancies and 65 of whom were born as a result of spontaneous multiple pregnancies were included in the study. Maternal age and morbidity, early rupture of membranes, birth weigth, gestational week, gender, APGAR score, hospitalization reasons in the neonatal period, requirement for intensive care, frequency of congenital anomaly, outpatient follow-up status, rehospitalization and socioeconomic levels were interrogated in the patients. Detailed physical examination and current height, weight and head circumference measurements were performed and the findings were placed in the growth curves of Neyzi et al. Ethics committee approval was received for this study from the ethics committee of Bakirköy Gynecology Obstetrics and Pediatrics Education and Research Hospital (12.10.2010; no:305). RESULTS: The rate of cesarean section, mean maternal age, the rate of chronic disease in the mother and the rate of maternal disease which occured during pregnancy were significantly higher in the in vitro fertilization group (p<0.05). While no difference was found in mean gestational age, birth weight, rate of hospitalization, time of hospitalization, frequency of follow-up in the intensive care unit, rates of congenital anomaly and rehospitalization, APGAR score in the 5(th) minute was significantly higher in the in vitro fertilization group. The socioeconomical score was not different between the groups, but the in vitro fertilization group presented more regularly for outpatient follow-up visits. Height, head circumference measurements and mean current weight were found to be significantly higher in the in vitro fertilization group (p<0.05). CONCLUSION: The fact that there was no difference in the rate of hospitalization, time of hospitalization, frequency of follow-up in the intensive care unit, rates of congenital anomaly and rehospitalization was attributed to the fact that the study and control groups were composed of only multiple pregnancies and preterms.

Evaluation of growth in very low birth weight preterm babies.

AIM: The aim of this study was to evaluate physical growth of very low birth weight (VLBW) preterm babies at a mean age of three years and to investigate the factors which affected growth. MATERIAL AND METHODS: The factors including maternal problems, prenatal problems, early neonatal problems, nutrition, familial socioeconomical status and presence of chronic disease which affected catch-up growth in terms of height and weight in VLBW infants followed up in the neonatal intensive care unit (NICU) of our hospital were examined. The target height formula was used in assessment of growht in height and the contribution of genetic properties was investigated. The points of the subjects on the growth curve were plotted according to the Percentile Curve of the Turkish Children prepared by Neyzi et al. The states of the subjects with and without intrauterine growth retardation (were compared. The study was intitiated after obtaining approval from the ethics committeee of our hospital (100/25.10.2005). RESULTS: One hundred and seventeen preterm babies (57 females and 60 males) with a mean adjusted age of 35.8±2.39 80 of whom were appropriate for gestational age (AGA), 28 of whom were symmetrical (small gestational age) SGA and 9 of whom were asymmetrical SGA were included in the study. The mean gestational age (GA) was found to be 31±2.16 weeks and the mean birth weight (BW) was found to be 1271±226 g. The mean current height was found to be 92.06±4.90 cm. The mean weight was found to be 12.98±1.94 kg. The mean target height was calculated to be 163.66±8.1 cm (157.20 cm for the girls and 170.20 cm for the boys). It was found that 15 preterm babies (12.8%) could not achieve the target height (girls: 6%, boys: 6.8%). The risk factors related with failure to achieve target height were found to include ventilator treatment, presence of chronic disease, advanced stage intracranial bleeding (ICB), posthemorrhagic hydrocephalus, absence of breastfeeding, failure to sit at the table with the family and malnutrition. The maternal age, early rupture of membranes (PROM), preeclampsia, smoking, early neonatal problems, gender, being AGA and SGA, gestational age, birth weight and socioeconomical level were statistically insignificant in terms of achieving target height (p>0.05). CONCLUSIONS: If very low birth weight preterm babies have no chronic disease and condition leading to neurodevelopmental retardation and if they are breastfed early and continuously (0-24 months), they can achieve catch-up growth similar to term babies. In examination of growth in terms of height, using target height may be more appropriate to shown the genetic potential.

Bacteremia due to Achromobacter xylosoxidans in neonates: clinical features and outcome

OBJECTIVE: We report an outbreak of Achromobacter xylosoxidans at a neonatal intensive care unit. We aimed to present clinical, laboratory and treatment data of the patients. Materials and METHODS: All consecutive episodes of bacteremia due to A. xylosoxidans at our neonatal intensive care unit, beginning with the index case detected at November 2009 until cessation of the outbreak in April 2010, were evaluated retrospectively. RESULTS: Thirty-four episodes of bacteremia occurred in 22 neonates during a 6-month period. Among the affected, 90% were preterm newborns with gestational age of 32 weeks or less and 60% had birth weight of 1000 g or less. Endotracheal intubation, intravenous catheter use, total parenteral nutrition and prolonged antibiotic therapy were the predisposing conditions. Presenting features were abdominal distention, thrombocytopenia and neutropenia. The mortality rate was 13.6% and the majority of isolates were susceptible to piperacillin-tazobactam, carbapenems and trimethoprim-sulfametoxazole, and resistant to gentamycin. More than half were breakthrough infections. Despite intensive efforts to control the outbreak by standard methods of hand hygiene, patient screening and isolation, containment could be achieved only after the neonatal intensive care unit was relocated. The investigation was not able to single out the source of the outbreak. CONCLUSION: A. xylosoxidans has the potential to cause serious infections in premature babies. More studies are needed to determine the importance of different sources of infection in hospital units.

Bacteremia due to Achromobacter xylosoxidans in neonates: clinical features and outcome.

OBJECTIVE: We report an outbreak of Achromobacter xylosoxidans at a neonatal intensive care unit. We aimed to present clinical, laboratory and treatment data of the patients. MATERIALS AND METHODS: All consecutive episodes of bacteremia due to A. xylosoxidans at our neonatal intensive care unit, beginning with the index case detected at November 2009 until cessation of the outbreak in April 2010, were evaluated retrospectively. RESULTS: Thirty-four episodes of bacteremia occurred in 22 neonates during a 6-month period. Among the affected, 90% were preterm newborns with gestational age of 32 weeks or less and 60% had birth weight of 1000g or less. Endotracheal intubation, intravenous catheter use, total parenteral nutrition and prolonged antibiotic therapy were the predisposing conditions. Presenting features were abdominal distention, thrombocytopenia and neutropenia. The mortality rate was 13.6% and the majority of isolates were susceptible to piperacillin-tazobactam, carbapenems and trimethoprim-sulfametoxazole, and resistant to gentamycin. More than half were breakthrough infections. Despite intensive efforts to control the outbreak by standard methods of hand hygiene, patient screening and isolation, containment could be achieved only after the neonatal intensive care unit was relocated. The investigation was not able to single out the source of the outbreak. CONCLUSION: A. xylosoxidans has the potential to cause serious infections in premature babies. More studies are needed to determine the importance of different sources of infection in hospital units.

Neonatal bacterial meningitis in Turkey: epidemiology, risk factors, and prognosis.

INTRODUCTION: We aimed to determine the incidence, etiology, risk factors and outcome of bacterial meningitis in neonates. METHODOLOGY: Neonates who developed bacterial meningitis between 2003 and 2010 in a tertiary hospital in Turkey were included in the study. Patients born in our hospital were defined as Group 1 and patients referred from other centres were defined as Group 2. Patients with evidence of congenital infections or central nervous system malformations were excluded. Demographic features, delivery type, time of onset of meningitis, co-morbidities, clinical features, blood and cerebrospinal fluid (CSF) analysis, cranial sonographic findings, and outcome of patients were recorded. RESULTS: The study comprised 325 meningitis cases identified from 38,023 hospitalised patients in the neonatology unit among 11,8091 live births. Mean gestational age, birth weight, and hospital stay were 36.8 ± 3.7 weeks, 2.480 ± 924 g, and 26 ± 12.4 days, respectively. Almost half (48%) of the patients were diagnosed in the first seven postnatal days and 52% at 8-30 days after birth. CSF culture findings were positive in 59 (18%) patients (28 in Group 1 and 31 in Group 2). Gram-positive bacteria were the responsible agents in 30 (51%) patients, whereas 26 (44%) patients had Gram-negative bacterial meningitis and 3 (5%) had Candida meningitis. Gram-negative bacteria were predominant in Group 1 whereas Gram positive bacteria were predominant in Group 2. Transfontanel ultrasonography revealed pathologic findings in 17.5% of patients. The total mortality rate was 2.5%. CONCLUSIONS: This large-scale study provides essential information about the etiology, characteristics, and outcome of neonatal bacterial meningitis in Turkey.

A rare cause of perinatal asphyxia: maternal carbon monoxide poisoning.

Carbon monoxide (CO) intoxication has serious adverse effects to the mother and fetus and a result of intrauterine hypoxia, it leads to fetal death or severe neurological sequelae. In this article, a preterm infant who was acutely exposed to CO at the 33rd weeks of gestation before delivery was presented. The baby was delivered by emergent cesarean section at the 34th weeks of gestation due to findings of fetal distress and he had severe hypoxic ischemic encephalopathy leading to death. Results and treatment modalities of CO poisoning during pregnancy were reviewed.