Neonatal Outcomes of Critical Congenital Heart Defects: A Multicenter Epidemiological Study of Turkish Neonatal Society : Neonatal Outcomes of CCHD.

Critical congenital heart disease (CCHD) is one of the leading causes of neonatal and infant mortality. We aimed to elucidate the epidemiology, spectrum, and outcome of neonatal CCHD in Türkiye. This was a multicenter epidemiological study of neonates with CCHD conducted from October 2021 to November 2022 at national tertiary health centers. Data from 488 neonatal CCHD patients from nine centers were entered into the Trials-Network online registry system during the study period. Transposition of great arteria was the most common neonatal CHD, accounting for 19.5% of all cases. Sixty-three (12.9%) patients had extra-cardiac congenital anomalies. A total of 325 patients underwent cardiac surgery. Aortic arch repair (29.5%), arterial switch (25.5%), and modified Blalock-Taussig shunt (13.2%). Overall, in-hospital mortality was 20.1% with postoperative mortality of 19.6%. Multivariate analysis showed that the need of prostaglandin E1 before intervention, higher VIS (> 17.5), the presence of major postoperative complications, and the need for early postoperative extracorporeal membrane oxygenation were the main risk factors for mortality. The mortality rate of CCHD in our country remains high, although it varies by health center. Further research needs to be conducted to determine long-term outcomes for this vulnerable population.

Cardiac effects of multisystem inflammatory syndrome in children: One-year follow-up.

AIM: Cardiovascular involvement is common among children with multisystem inflammatory syndrome (MIS-C) and can cause shock and death. In this study, we evaluated the early and long-term cardiac effects of MIS-C. METHODS: In this observational cohort study, we included all children treated for MIS-C from October 2020 to November 2021 in the Department of Paediatric Infectious Disease at Cukurova University School of Medicine Hospital. The patients underwent serial echocardiographical evaluation during hospitalisation and at 1, 3, 6 and 12 months after discharge. The patients were evaluated using Holter monitorisation between 4 and 6 months and using cardiac magnetic resonance imaging at 6 months and thereafter. RESULTS: Twenty-six patients diagnosed with MIS-C and with a median age of 84 months were included. Cardiac involvement was found in 19 (73.1%) patients. At initial echocardiographic evaluation, the mean ejection fraction value of the patients was 56.7% (range: 30-75). Coronary artery dilatation was detected in two (7.7%) patients, and mitral regurgitation persisted in only one patient by month 3. Treatment was started in two (7.7%) patients due to ventricular arrhythmia. Cardiac magnetic resonance imaging was performed in 13 (50%) patients at a median of 6 months (range: 5-9). The cardiac magnetic resonance imaging findings were consistent with possible interstitial fibrosis in two (7.7%) patients. CONCLUSION: Our results showed that cardiac involvement of patients improved rapidly with treatment, as indicated by previous studies. However, during the 1-year follow-up, frequent extraventricular systole was detected in two patients, one of whom initially did not show cardiac involvement. Moreover, possible interstitial fibrosis was detected in the cardiac magnetic resonance imaging (MRI) evaluation of two patients. In particular, we believe that these findings may be useful to evaluate critically ill paediatric patients and patients with severely low EF with cardiac MRI in their follow-up.

Infective endocarditis in childhood: a single-centre experience of 26 years.

The aim of this study was to present the clinical and microbiological characteristics of patients with infective endocarditis.A retrospective evaluation was made of patients diagnosed with infective endocarditis between 1995 and 2021. The clinical and laboratory characteristics of the patients were recorded together with conditions constituting a risk for the development of endocarditis, treatment, and surgical outcomes.Evaluation was made of 68 patients with a mean age of 7.3 years (3 months-17 years), diagnosed with infective endocarditis. An underlying cause of CHD was determined in 47 (69%) patients and rheumatic valve disease in 3 (4.4%). There was no structural heart disease in 18 (26%) patients of whom 13 of them had other risk factors. A causative organism was found in 41 (60%) cases, and the microorganism most often determined was viridans group streptococcus. No difference was determined between the patients diagnosed before and after 2007 in respect of the frequency of viridans streptococcus (p > 0.05). Septic emboli were seen in 18 (26%) patients, of which 17 required surgical treatments. In 5 of 11 (16.6%) patients with mortality, the agent was S. aureus. Of the total 28 (41%) patients were evaluated as hospital-acquired endocarditis. The most frequently determined agents in this group were staphylococcus epidermidis and S. aureus.Although CHDs continue to be the greatest risk factor for endocarditis, there is an increasing frequency of endocarditis in patients with no structural heart disease. Mortality rates are still high in infective endocarditis, especially in S. aureus endocarditis.

Clinical features and echocardiographic findings of isolated foramen ovale restriction in foetuses.

Isolated restrictive foramen ovale (rFO) without complex heart defects is a rare pathology. There may be difficulties in managing this situation, which can lead to right heart enlargement, tricuspid regurgitation and hydrops findings in the foetus. We conducted a retrospective analysis of 8451 foetuses. 7883 (93.2%) had a structurally normal heart or minor heart disease, 18 (0.22%) of which had a diagnosis of isolated rFO. Nine patients with neonatal echocardiographic examination were included in the study. In 8 (88.8%) patients, it was stated that a decision to give birth should be made at the time of presentation. Evaluating postpartum echocardiographic examinations, 7 (77.7%) patients had normal or minor defects. The decision of delivery made at the right time during follow-up is critical to determine the prognosis.IMPACT STATEMENTWhat is already known on this subject? The data about the prenatal diagnosis of isolated rFO is limited.What the results of this study add? We conducted a retrospective analysis of 8451 foetuses. 7883 (93.2%) foetuses had a structurally normal heart or minor heart disease, 18 (0.22%) of which had a diagnosis of rFO. Nine patients with foetal and postnatal follow-up and echocardiographic findings were examined in the study. The group's median gestational age at admission was 35.0 weeks (range: 27.0-39.0 weeks). The delivery decision was made in 8 (88.8%) patients at the time of admission. Evaluating postpartum echocardiographic examinations, 7 (77.7%) patients had normal or minor defects. Additionally, one patient was diagnosed with cardiomyopathy, and the other patient was diagnosed with functional pulmonary atresia. No death occurred in any foetus during follow-up.What the implications are of these findings for clinical practice and/or further research? Isolated rFO, a rare condition in the foetus, is generally well-tolerated in foetal life. However, the right heart enlargement, tricuspid regurgitation, or hydrops findings can be seen in patients. The decision of delivery made at the right time during follow-up is critical to determine the prognosis.

Acute rheumatic fever in south-east of Turkey: clinical features and epidemiological evaluation of the patients over the last 25 years.

This study evaluates clinical and epidemiological features of acute rheumatic fever using the data of last 25 years in our hospital in south-east of Turkey. The medical records of 377 patients with acute rheumatic fever admitted to Pediatric Cardiology Department of Çukurova University during 1993-2017 were retrospectively analysed. Two hundred and six patients were admitted between 1993 and 2000, 91 between 2001 and 2008, and 80 between 2009 and 2017. The largest age group (52%) were between 9 to 12 years of age and approximately two-thirds of the patients presented in the spring and winter seasons (62.8%). Among the major findings, the most common included carditis 83.6% (n = 315), arthritis at 74% (n = 279), Sydenham's chorea at 13.5% (n = 51), and only two patients (0.5%) had erythema marginatum and two patients (0.5%) had subcutaneous nodule. Carditis was the most common manifestation observed in 315 patients (83.6%). The most commonly affected valve was the mitral valve alone (54.9%), followed by a combined mitral and aortic valves (34%) and aortic valve alone (5.7%). Of the patients with carditis, 48.6% (n = 153) had mild carditis, of which 45 had a subclinical. Sixty-two patients (19.7%) had moderate and 100 patients (31.7%) had severe carditis. At the follow-up, 2 patients died and 16 patients underwent valve surgery. Twenty-eight (7.4%) patients' valve lesions were completely resolved. Conclusion: Although the incidence of acute rheumatic fever decreased, it still is an important disease that can cause serious increases in morbidity and mortality rates in our country.

Myocarditis and Early Markers of Cardiac Response Associated with Scorpion Stings in Children.

INTRODUCTION: A clinical course ranging from mild local findings to life-threatening systemic findings may occur after scorpion stings. The purpose of this study was to identify priority markers indicating scorpion sting-related cardiac involvement. METHODS: Our study was performed between July 2014, and September 2015 in the Çukurova University medical faculty pediatric emergency department, in Adana, Turkey. Patients admitted with scorpion sting-related cardiac involvement and a control group consisting of patients with no scorpion sting-related cardiac involvement were included in the study. Troponin I at time of presentation and at 6 and 24 h, N-terminal prohormone of brain natriuretic peptide (NTproBNP), ejection fraction as determined by echocardiography at 24 h, and peak and end of T wave (Tp-e) and Tp-e/QTc ratios with echocardiography at 24 h were evaluated. RESULTS: A patient group consisting of 7 cases of scorpion envenomation-related myocarditis and a control group of 30 cases of scorpion intoxication without myocarditis findings were enrolled. Statistically significantly high glucose, white blood cell values, creatine kinase MB, troponin I, and NTproBNP values were identified in the scorpion sting-related myocarditis group (P<0.05). Ejection fractions determined by echocardiography at time of presentation were significantly lower in the patients with myocarditis compared with the control group (P<0.05). A statistically significant difference was identified between Tp-e/corrected QT interval (QTc) ratios investigated in DI and V2 derivations in patient and control group echocardiograms (P<0.05). CONCLUSIONS: We think that use can be made of NTproBNP in addition to echocardiography and troponin I in the early diagnosis of scorpion sting-related myocarditis and that Tp-e and Tp-e/QTc ratios identified via echocardiography can be used as early markers; however, further studies with larger numbers are needed to confirm this.

Neurofibromatosis type 1 and cardiac manifestations.

OBJECTIVE: Cardiac manifestations of neurofibromatosis type 1 (NF1) may include hypertension, congenital heart disease, and hypertrophic cardiomyopathy. The aim of this study was to evaluate cardiac abnormalities in patients with NF1. METHODS: Sixty-five NF1 patients (mean age: 9±4.48 years) were retrospectively studied. Standard electrocardiography and echocardiography were performed in all patients. RESULTS: Cardiac abnormalities were found in 11 of the 65 patients (15.3%). Five patients had mitral valve regurgitation, 2 patients had secundum atrial septal defect, 1 patient had pulmonary valvular stenosis, 1 patient had ventricular septal defect, 1 patient had tricuspid valve regurgitation, and 1 patient had aortic valve regurgitation. CONCLUSION: Cardiac abnormalities have potential long-term hemodynamic consequences that justify an early diagnosis. Thus, for any patient with NF1, a cardiologic assessment is mandatory at the time of diagnosis and with regular follow-up intervals.

Effect of antiepilepsy drug therapy on ventricular function in children with epilepsy: a tissue Doppler imaging study.

Impaired cardiac myocardial function may contribute to the risk for sudden unexpected death of a patient with epilepsy. This study aimed to investigate the effect of antiepilepsy drugs (AEDs) on cardiac function in pediatric epileptic patients using standard and tissue Doppler imaging (TDI) echocardiography. This hospital-based, prospective cross-sectional study investigated 52 epileptic children (mean age 9.3 ± 3.1 years) treated with AEDs (duration 2.4-10.0 years) and 36 healthy children (mean age 9.5 ± 4.0 years). In the epilepsy group, standard echocardiography showed increased left ventricular (LV) end-diastolic and end-systolic diameters, an increased LV mass index, and preserved ejection fraction. The patients also exhibited increased mitral peak A-wave velocity and mitral E-wave deceleration time as well as a decreased mitral E/A ratio. The E/Em ratio was significantly higher in the epilepsy group (5.6 ± 1.2) than in the control group (5.2 ± 1.1) (p = 0.016). In the epilepsy group, TDI showed an increased isovolumetric relaxation time and myocardial performance index (MPI). It also exhibited decreased early diastolic velocity (Em) and a decreased mitral annular displacement index in these patients. There were positive correlations between the LV lateral wall MPI (r = 0.231), septal MPI (r = 0.223), and LV mass index (p < 0.05) but no correlation with the duration of AED treatment. The authors detected subclinical ventricular dysfunction associated with AEDs at a preclinical stage. They suggest that TDI can be useful for determining the short- and long-term cardiac effects of AEDs.

Short- and mid-term results of xenograft-bovine pericardial patch in the repair of intracardiac defects: final results of a single-centre study.

INTRODUCTION: A variety of patch materials have been used in the repair of intracardiac defects. We evaluated the short- and mid-term clinical and echocardiography results of glutaraldehyde-preserved bovine pericardium patches used to repair intracardiac defects in our clinic. METHODS AND RESULTS: This study examines the short- (up to 30 days post-operatively) and mid-term (up to 24 months post-operatively) results of 533 patients with intracardiac defects who underwent surgical correction with glutaraldehyde-preserved bovine pericardium patches between 2004 and 2010 at a university clinic. Short- and mid-term post-operative echocardiographic studies showed no evidence of calcification, thrombus, or aneurysmal dilatation on the patch. Vegetation developed in two (0.37%) of the 533 patients. CONCLUSION: These results that have been obtained from a group of large number of patients imply that the glutaraldehyde-preserved bovine pericardium patches may be preferable in the closure of cardiac defects because of their low complication rates and ease of use.

Decline in Left Ventricular Early Systolic Function with Worsening Kidney Function in Children with Chronic Kidney Disease: Insights from the 4C and HOT-KID Studies.

INTRODUCTION: Adults with childhood-onset chronic kidney disease (CKD) have an increased risk of cardiovascular disease. First-phase ejection fraction (EF1), a novel measure of early systolic function, may be a more sensitive marker of left ventricular dysfunction than other markers in children with CKD. OBJECTIVE: To examine whether EF1 is reduced in children with CKD. METHODS: Children from the 4C and HOT-KID studies were stratified according to estimated glomerular filtration rate (eGFR). The EF1 was calculated from the fraction of left ventricular (LV) volume ejected up to the time of peak aortic flow velocity. RESULTS: The EF1 was measured in children ages 10.9 ± 3.7 (mean ± SD) years, 312 with CKD and 63 healthy controls. The EF1 was lower, while overall ejection fraction was similar, in those with CKD compared with controls and decreased across stages of CKD (29.3% ± 3.7%, 23.5% ± 4.5%, 19.8% ± 4.0%, 18.5% ± 5.1%, and 16.7% ± 6.6% in controls, CKD 1, 2, 3, and ≥ 4, respectively, P < .001). The relationship of EF1 to eGFR persisted after adjustment for relevant confounders (P < .001). The effect size for association of measures of LV structure or function with eGFR (SD change per unit change in eGFR) was greater for EF1 (ß = 0.365, P < .001) than for other measures: LV mass index (ß = -0.311), relative wall thickness (ß = -0.223), E/e' (ß = -0.147), and e' (ß = 0.141) after adjustment for confounders in children with CKD. CONCLUSIONS: Children with CKD exhibit a marked and progressive decline in EF1 with falling eGFR. This suggests that EF1 is a more sensitive marker of LV dysfunction when compared to other structural or functional measures and that early LV systolic function is a key feature in the pathophysiology of cardiac dysfunction in CKD.

Three patients resembling Teebi-Shaltout syndrome.

Teebi-Shaltout syndrome (TSS) was first reported by Teebi and Shaltout in 1989. This entity is proposed to be inherited in autosomal recessive manner. The clinical features include characteristic facial features, ectodermal dysplasia, camptodactyly, and caudal appendage. Only one additional paper reporting four additional cases has been published since the first description. Clinical features common to all previously affected individuals diagnosed with TSS are craniofacial, orodental-ectodermal, and skeletal. This report summarizes and discusses the findings of three additional patients from two unrelated families with findings similar to TSS. These findings may be present in a genetically and phenotypically heterogeneous group of disorders similar to TSS. Presence of consanguinity and similarly affected siblings of both genders suggests autosomal recessive inheritance.

Successful implantation of an intracardiac defibrillator in an infant with long QT syndrome and isolated noncompaction of the ventricular myocardium.

Congenital long QT syndrome (LQTS) is an inherited disorder characterized by QT prolongation and polymorphic ventricular tachycardia known as torsade de pointes. The underlying cellular mechanism is prolonged ventricular repolarization caused by mutations in genes encoding cardiac ion channels or membrane adaptors. The disease can be diagnosed at any age and, very rarely, it can be diagnosed prenatally or in the neonatal period. Isolated noncompaction of the ventricular myocardium (INCVM) is defined as the presence of prominent ventricular trabeculations and deep intertrabecular recesses within the endomyocardium. This report describes a newborn baby presenting with polymorphic ventricular tachycardia whose diagnosis was LQTS and INCVM. Ventricular tachycardia did not respond to medical treatment, and a transient epicardial pacemaker was inserted surgically on his 30th day of life for atrioventricular block and bradycardia. The transient epicardial pacemaker was upgraded to an epicardial intracardiac defibrillator on his 40th day. The concomitant occurrence of INCVM, LQTS, and atrioventricular block needs to be evaluated further.

Anomalous origin of coronary arteries from the pulmonary artery: A single-center experience.

Background: The aim of the study was to evaluate the clinical and diagnostic findings, treatment, and follow-up of cases of anomalous origin of coronary arteries from the pulmonary artery. Methods: Between January 1998 and June 2021, a total of 14 patients (5 males, 9 females; median age: 15 months; range, 3 to 156 months) diagnosed with anomalous origin of coronary arteries from the pulmonary artery were retrospectively analyzed. Demographic and clinical data of the patients, electrocardiographic, echocardiographic, angiographic, surgical, and follow-up findings were evaluated. Results: The most common symptoms were respiratory distress (n=6) and murmur (n=3). With the exception of three cases, all other patients were diagnosed by echocardiography in the first examination. Severe mitral valve insufficiency was detected in four patients and four other patients had moderate mitral insufficiency on echocardiography. Ejection fraction values ranged between 38 and 79%. Eleven patients underwent direct implantation of the coronary artery into the aorta, and three underwent a Takeuchi procedure. Mortality occurred in only one case. After surgery, mitral insufficiency and ejection fraction values improved. Median follow-up was 62 (range, 5 to 170) months and all patients were asymptomatic, except one who required redo surgery. Conclusion: Anomalous origin of the coronary arteries from the pulmonary artery is an uncommon congenital anomaly. Echocardiography is the main diagnostic tool. If all echocardiographic findings of the coronary anomaly are not investigated thoroughly, the diagnosis may be overlooked.

Temporary atrioventricular complete block that develops following the transcatheter closure of ventricular septal defect.

Atrioventricular (AV) block is a potential risk after transcatheter closure of perimembranous ventricular septal defect (VSD) with the Amplatzer perimembranous device. We present herein a case of a 6.5-year-old female patient who developed complete AV block six days after closure of VSD and recovered with steroid and salicylate treatment. It is important to be alert to the development of AV block after transcatheter perimembranous VSD closure. Very careful monitoring of rhythm is mandatory during the short- and long-term follow-up.

Mid-term results of patients following total surgical correction of tetralogy of Fallot.

The aim of this study was to evaluate the mid-term clinical status of patients following tetralogy of Fallot (TOF) repair. We performed a cross-sectional observational analysis of 57 postoperative TOF patients and 58 age-matched controls. Patients were examined with myocardial performance index (MPI), which was obtained by tissue Doppler imaging (TDI) in addition to the conventional methods. Compared with controls, patients had significantly higher right ventricular (RV) dimensions and volumes, RV MPI and left ventricular (LV) MPI, and significantly lower RV ejection fraction (EF), LV EF, exercise duration, and maximum heart rate with exercise (p<0.001 for all parameters). A positive correlation was found between RV MPI and LV MPI (r=0.541, p<0.001). There was a negative correlation between LV MPI and exercise duration (r=0.260, p=0.034). After total surgical correction of TOF, the patients had a good clinical status at the mid-term follow-up; decreased exercise capacity and impaired functions in both ventricles related to the degree of pulmonary regurgitation were found. By using MPI, impaired cardiac functions can identify such conditions before they become clinically symptomatic.

[Long term follow-up of 799 children with isolated ventricular septal defects]. / Izole ventriküler septal defektli 799 çocugun uzun süreli takibi.

OBJECTIVES: We evaluated long-term follow-up results and prognosis of pediatric patients with isolated ventricular septal defects (VSD). STUDY DESIGN: The study included 799 patients (368 girls, 431 boys; mean age at diagnosis 24.3±37.4 months; median 6 months) who were monitored by the pediatric cardiology department for VSD. The mean follow-up period was 32.8±30.3 months (median 20 months). RESULTS: The VSDs were classified as perimembranous (n=610, 76.4%), muscular (n=171, 21.4%), doubly committed subarterial (n=10, 1.3%), and multiple (n=8, 1%). Spontaneous closure rates were 42.7%, 13.1%, and 25% in muscular, perimembranous, and multiple VSDs, respectively, which corresponded to a mean age of 18.6±19.9 months (median 12 months) in muscular and 30.2±33.7 months (median 14.5 months) in perimembranous VSDs. Before 2 years of age, 78.1% of muscular and 58.6% of perimembranous VSDs underwent spontaneous closure. Of 256 defects (32%) that required surgical closure, 91.4% were of perimembranous location. The mean age at surgery was 38.8±49.1 months (median 11 months) for muscular, and 43.7±40.9 months (median 24 months) for perimembranous defects. During the follow-up period, the following complications were noted: aortic valve prolapse (0.7%), aortic regurgitation (0.6%), left ventricle-to-right atrium shunt (2.6%), subaortic ridge (3.7%), and infundibular stenosis (1.2%). Aortic regurgitation developed in eight patients (3.7%) after surgical closure. CONCLUSION: Our data on the natural course and prognosis of VSDs may be of relevance with respect to patients' age, defect type, and complications encountered in the follow-up period.

Prenatal Diagnosis, Associations and Outcome for Fetuses with Congenital Absence of the Pulmonary Valve Syndrome.

BACKGROUND: The aim of this study is to review the spectrum of the prenatally detected absent pulmonary valve syndrome and its outcome after diagnosis. METHODS: Clinical data and echocardiographic findings of 37 cases with a fetal diagnosis of absent pulmonary valve syndrome between 2008 and 2020 were analyzed in this retrospective multicenter study. RESULTS: Median gestational age at diagnosis was 25 weeks. Three subtypes of absent pulmonary valve syndrome were observed: (1) with tetralogy of Fallot (n=30; 81.0%); (2) absent pulmonary valve syndrome with intact ventricular septum (n=5; 13.5%); (3) with complete atrioventricular septal defect (n=2; 5.4%). In contrast to 7/25 fetuses (28%) with tetralogy of Fallot-absent pulmonary valve syndrome who had a patent ductus arteriosus, all 5 fetuses with absent pulmonary valve syndrome-intact ventricular septum had a patent ductus arteriosus (P < .001). No significant difference was found between the z-scores of pulmonary artery branches in fetuses with or without patent ductus arteriosus (P > .05). The analysis did not reveal any correlation between gestational week and z-scores of pulmonary artery, pulmonary artery branches (right pulmonary artery, left pulmonary artery), and ratio of aorta/pulmonary artery ratio. The echocardiographic measurements of survivors did not differ significantly from non-survivors (P > .05). Extracardiac anomalies were observed in 8/37 fetuses (21.6%). The incidence of extracardiac anomaly was significantly higher in cases of tetralogy of Fallot-absent pulmonary valve syndrome (P < .05). Overall, 9 fetuses (24%) had genetic anomalies. All 6 fetuses (20%) with 22q11.2 microdeletion were within the tetralogy of Fallot-absent pulmonary valve syndrome group. Overall survival after initial diagnosis in the total cases was 36.6% (11/30), with 9 of 30 (30%) tetralogy of Fallot-absent pulmonary valve syndrome cases and 2 of 5 (40%) absent pulmonary valve syndrome-intact ventricular septum cases. CONCLUSIONS: In this largest series of absent pulmonary valve syndrome, extracardiac, and chromosomal anomalies were found to be a common occurrence. The risk of 22q11.2 microdeletion was higher in tetralogy of Fallot cases at 40%. The sizes of the pulmonary artery and its branches and the aorta had no correlation of high mortality antenatally or after birth, which were 63.4% and 47.7%, respectively.

Brain natriuretic peptide levels in pediatric cancer patients with febrile neutropenia.

Brain natriuretic peptide (BNP) is considered as a prognostic marker in patients with sepsis, but no data are available on BNP in pediatric cancer patients with febrile neutropenia (FN). Twenty-five pediatric cancer patients with FN were included in this study. Serum BNP level was measured. The mean BNP level was 330.8 ± 765.3 pg/mL (5.9-3806 pg/mL). BNP levels of 12 patients were found over the normal level. High BNP levels were related to some conditions of the patients, and these were statistically significant (P < .05). These conditions were required erythrocyte suspension, had pneumonia, time stayed in hospital, and neutropenia time. When regression test was done, required erythrocyte suspension for anemia and had pneumonia were found to be statistically significant. In conclusion, this is one of the first studies on BNP levels in pediatric cancer patients with FN. However, further studies with large sample sizes are needed to confirm the results and provide new data about this issue.

Beneficial effects of terlipressin in pediatric cardiac arrest.

OBJECTIVE: : Vasopressin and its analog, terlipressin (TP), are potent vasopressors that may be useful therapeutic agents in the treatment of cardiac arrest (CA), septic and catecholamine-resistant shock, and esophageal variceal hemorrhage. The American Heart Association 2000 guidelines recommend its use for adult ventricular fibrillation arrest, and the American Heart Association 2005 guidelines note that it may replace the first or second epinephrine dose. There is little reported experience with TP in cardiopulmonary resuscitation (CPR) of children. The purpose of this retrospective case series was to report successful return of spontaneous circulation after the rescue administration of vasopressin after prolonged CA and failure of conventional CPR, advanced life support, and epinephrine therapy in children. METHODS: : Nine pediatric patients with asystole, aged 11 months to 14 years, who experienced 12 episodes of refractory CA and did not respond to conventional therapy. Terlipressin was administered as intravenous bolus doses of 20 mcg/kg to standard cardiopulmonary resuscitation. RESULTS: : Return of spontaneous circulation was monitored and achieved in 6 of the 12 episodes. The mean duration of CPR was 24.8 minutes in these 12 episodes of CA with TP administration, with a range of 10 to 50 minutes (median, 23 minutes). Five survivors were discharged home without sequelae and with good neurologic status (score 1 by the pediatric cerebral performance category). CONCLUSIONS: : The combination of TP to epinephrine during CPR may have a beneficial effect in children with CA. However, the recommendations for its use in the pediatric literature are based on limited clinical data.

Morquio A syndrome and effect of enzyme replacement therapy in different age groups of Turkish patients: a case series.

BACKGROUND: This case series includes longitudinal clinical data of ten patients with Morquio A syndrome from south and southeastern parts of Turkey, which were retrospectively collected from medical records. All patients received enzyme replacement therapy (ERT). Clinical data collected included physical appearance, anthropometric data, neurological and psychological examinations, cardiovascular evaluation, pulmonary function tests, eye and ear-nose-throat examinations, endurance in the 6-min walk test and/or 3-min stair climb test, joint range of motion, and skeletal investigations (X-rays, bone mineral density). RESULTS: At the time of ERT initiation, two patients were infants (1.8 and 2.1 years), five were children (3.4-7.1 years), and three were adults (16.5-39.5 years). Patients had up to 4 years follow-up. Most patients had classical Morquio A, based on genotypic and phenotypic data. Endurance was considerably reduced in all patients, but remained relatively stable or increased over time in most cases after treatment initiation. Length/height fell below normal growth curves, except in the two infants who started ERT at ≤ 2.1 years of age. All patients had skeletal and/or joint abnormalities when ERT was started. Follow-up data did not suggest improvements in skeletal abnormalities, except in one of the younger infants. Nine patients had corneal clouding, which resolved after treatment initiation in the two infants, but not in the other patients. Hepatomegaly was reported in seven patients and resolved with treatment in five of them. Other frequent findings at treatment initiation were coarse facial features (N = 9), hearing loss (N = 6), and cardiac abnormalities (N = 6). Cardiac disease deteriorated over time in three patients, but did not progress in the others. CONCLUSIONS: Overall, this case series with Morquio A patients confirms clinical trial data showing long-term stabilization of endurance after treatment initiation across ages and suggest that very early initiation of ERT optimizes growth outcomes.