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BACKGROUND: The prevalence of heart failure is constantly increasing in both children and adults. End-stage heart failure in children unresponsive to medical therapy has limited treatment options. Surgical options include heart transplantation or implantation of durable ventricular assist devices (VADs). To start the VAD program, it was necessary to train core team members, invite experienced proctors and adjust the organizational approach. METHODS: We present our first seven pediatric patients who underwent a VAD implantation with primary indication end-stage dilated cardiomyopathy. RESULTS: The median age on implant was four and a half years and the median duration of VAD support was 39 days with long term survival achieved in three patients. The causes of death were multiorgan failure, thromboembolic events, sepsis, and low cardiac output syndrome. Ischemic stroke was the reason for successful neurointervention during VAD support in two patients. CONCLUSIONS: To establish a VAD program, numerous specialties must be included with adequate training and learning for all team members.
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Insuficiencia Cardíaca , Trasplante de Corazón , Corazón Auxiliar , Accidente Cerebrovascular Isquémico , Adulto , Humanos , Niño , Insuficiencia Cardíaca/cirugía , Insuficiencia MultiorgánicaRESUMEN
INTRODUCTION: Extracorporeal membrane oxygenation (ECMO) is an important treatment option for organ support in respiratory insufficiency, cardiac failure, or as an advanced tool for cardiopulmonary resuscitation. Reports on pediatric ECMO use in our region are lacking. METHODS: This study is a retrospective review of all pediatric cases that underwent a veno-arterial (VA) or veno-venous (VV) ECMO protocol between November 2009 and August 2020 at the Department of Pediatrics, University Hospital Center Zagreb, Croatia. RESULTS: Fifty-two ECMO runs identified over the period; data were complete for 45 cases, of which 23 (51%) were female, and median age was 8 months. Thirty-eight (84%) patients were treated using the VA-and 7 (16%) using VV-ECMO. The overall survival rate was 51%. Circulatory failure was the most common indication for ECMO (N = 38, 84%), and in 17 patients ECMO was started after cardiopulmonary resuscitation (E-CPR). Among survivors, 74% had no or minor neurological sequelae. Variables associated with poor outcome were renal failure with renal replacement therapy (p < .001) and intracranial injury (p < .001). CONCLUSION: Overall survival rate in our cohort is comparable to the data published in the literature. The use of hemodialysis was shown to be associated with higher mortality. High rates of full neurological recovery among survivors are a strong case for further ECMO program development in our institution.
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Oxigenación por Membrana Extracorpórea , Insuficiencia Cardíaca , Humanos , Niño , Femenino , Lactante , Masculino , Oxigenación por Membrana Extracorpórea/métodos , Croacia , Estudios Retrospectivos , Tasa de SupervivenciaRESUMEN
Background: The use of high-sensitive cardiac troponin T (hsTnT) in urine as a marker of cardiac damage in children has not yet been reported. Elimination of cardiac troponins is dependent on renal function; persistently increased serum hsTnT concentrations were observed among individuals with impaired renal function. The aim of this study was to investigate serum and urine hsTnT levels and its correlation in infants and children younger than 24 months of age after cardiac surgery. Methods: This study was conducted on 90 infants and children under 24 months of age who were divided into three groups. The experimental group consisted of patients with intracardiac surgery of ventricular septal defect (VSD), first control group consisted of infants with extracardiac formation of bidirectional cavopulmonary connection (BCPC), and the second control group consisted of healthy children. Troponin T values ââwere determined in serum and urine at five time points: the first sample was taken on the day before cardiac surgery (measure 0) and the other four samples were taken after the surgery; immediately after (measure 1), on the first (measure 2), third (measure 3), and fifth postoperative day (measure 5). The first morning urine was sampled for determining the troponin T in the control group of healthy infants. Results: A positive correlation between troponin T values in serum and urine was found. Urine hsTnT measured preoperatively in children undergoing BCPC surgery was higher (median 7.3 [IQR 6.6-13.3] ng/L) compared to children undergoing VSD surgery (median 6.5 [IQR 4.4-8.9] ng/L) as well as to healthy population (median 5.5 [IQR 5.1-6.7] ng/L). After logarithmic transformation, there was no statistically significant difference in urine hsTnT concentration between the groups at any point of measurement preoperatively or postoperatively. Statistically significant negative correlation was found between serum and urine hsTnT concentrations and glomerular filtration rate estimated by creatinine clearance. Patients who underwent surgical repair of VSD had significantly higher concentrations of troponin T in serum on the first three postoperative measurements compared to those who had BCPC surgery. Conclusions: According to the results of this study, renal function after cardiac surgery appears to have a major effect on the urinary hsTnT concentrations, and we cannot conclude that this is an appropriate marker for the assessment of postoperative myocardial damage in children. Nevertheless, more research is needed to reach a better understanding of the final elimination of cardiac troponins in children.
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Our aim was to evaluate biochemical markers in plasma (NGAL, CysC) and urine (NGAL, KIM-1) in children's early onset of acute kidney injury after congenital heart defect surgery using cardiopulmonary bypass. This study prospectively included 100 children with congenital heart defects who developed AKI. Patients with acute kidney injury had significantly higher CysC levels 6 and 12 h after cardiac surgery and plasma NGAL levels 2 and 6 h after cardiac surgery. The best predictive properties for the development of acute kidney injury are the combination (+CysCpl or +NGALu) after 12 h and a combination (+CysCpl and +NGALu) 6 and 24 h after cardiac surgery. We showed that plasma CysC and urinary NGAL could reliably predict the development of acute kidney injury. Measurement of early biochemical markers in plasma and urine, individually and combination, may predict the development of cardiac surgery-associated acute kidney injury in children.
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Lesión Renal Aguda , Procedimientos Quirúrgicos Cardíacos , Lesión Renal Aguda/diagnóstico , Lesión Renal Aguda/etiología , Proteínas de Fase Aguda , Biomarcadores , Procedimientos Quirúrgicos Cardíacos/efectos adversos , Niño , Femenino , Humanos , Lipocalina 2 , Masculino , Valor Predictivo de las Pruebas , Proteínas Proto-OncogénicasRESUMEN
Specific protocols define eligibility, conditioning, donor selection, graft composition and prophylaxis of graft vs. host disease for children and young adults undergoing hematopoietic stem cell transplant (HSCT). However, international protocols rarely, if ever, detail supportive care, including pharmaceutical infection prophylaxis, physical protection with face masks and cohort isolation or food restrictions. Supportive care suffers from a lack of scientific evidence and implementation of practices in the transplant centers brings extensive restrictions to the child's and family's daily life after HSCT. Therefore, the Board of the Pediatric Diseases Working Party (PDWP) of the European Society for Blood and Marrow Transplantation (EBMT) held a series of dedicated workshops since 2017 with the aim of initiating the production of a set of minimal recommendations. The present paper describes the consensus reached within the field of infection prophylaxis.
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BACKGROUND: Candidemia is one of the leading causes of bloodstream infections in the pediatric intensive care unit (PICU). The aim of this study was to define characteristics and risk factors for candidemia in the PICU setting and propose a predictive model to identify the patients at risk. METHODS: This was a retrospective matched case-control study in the PICU during a 9-year period. Patients with candidemia were studied and matched with control patients without candidemia. Univariate analysis was performed for potential risk factors and multivariate analysis was conducted to determine the prediction score for candidemia. RESULTS: Forty-two cases of candidemia were matched with 84 control patients. Candida parapsilosis was the most common (71.4%) species. Risk factors independently associated with candidemia were: the use of >2 antibiotics in a maximum period of 4 weeks before the candidemia (odds ratio [OR]: 10.59; 95% confidence interval [CI]: 2.05-54.83), a previous bacterial infection in a maximum period of 4 weeks before the candidemia (OR: 5.56; 95% CI: 1.44-21.5) and the duration of PICU stay of >10 days (OR: 4.22; 95% CI: 1.02-17.41). The proposed predictive scoring system has a sensitivity of 95.24%, specificity of 76.12%, OR 64.0, 95% CI 14.2-288.6, the positive predictive value of 66.67% and the negative predictive value of 96.97%. CONCLUSIONS: Previously reported risk factors for candidemia have been confirmed and some new have been detected. The presented scoring system can help identify patients who would benefit from prophylactic antifungal therapy.
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Candida/patogenicidad , Candidemia/epidemiología , Hospitales Universitarios/estadística & datos numéricos , Unidades de Cuidado Intensivo Pediátrico/estadística & datos numéricos , Adolescente , Antifúngicos/uso terapéutico , Candidemia/tratamiento farmacológico , Candidemia/etiología , Estudios de Casos y Controles , Niño , Preescolar , Croacia/epidemiología , Humanos , Lactante , Recién Nacido , Oportunidad Relativa , Estudios Retrospectivos , Factores de RiesgoRESUMEN
AIM: To investigate the risk factors and the outcomes of extracorporeal membrane oxygenation (ECMO) in pediatric patients treated at the University Hospital Center Zagreb, the largest center in Croatia providing pediatric ECMO. METHODS: This retrospective study enrolled all the pediatric patients who required E-CPR from 2011 to 2019. Demographic data, cardiac anatomy, ECMO indications, ECMO complications, and neurodevelopmental status at hospital discharge were analyzed. RESULTS: In the investigated period, E-CPR was used in 16 children, and the overall survival rate was 37.5%. Six patients were in the neonatal age group, 5 in the infant group, and 5 in the "older" group. There was no significant difference between the sexes. Four patients had an out-of-hospital arrest and 12 had an in-hospital arrest. Twelve out of 16 patients experienced renal failure and needed hemodialysis, with 4 out of 6 patients in the survivor group and 8 out of 10 in the non-survivor group. Survivors and non-survivors did not differ in E-CPR duration time, lactate levels before ECMO, time for lactate normalization, and pH levels before and after the start of ECMO. CONCLUSION: The similarity of our results to those obtained by other studies indicates that the ECMO program in our hospital should be maintained and improved.
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Reanimación Cardiopulmonar , Oxigenación por Membrana Extracorpórea , Niño , Croacia/epidemiología , Humanos , Lactante , Estudios Retrospectivos , Tasa de Supervivencia , Resultado del TratamientoRESUMEN
Allogeneic haematopoietic stem cell transplantation (HSCT) is indicated in children with high-risk, relapsed or refractory acute lymphoblastic leukaemia (ALL). HLA-matched grafts from cord blood and stem cell repositories have allowed patients without suitable sibling donors to undergo HSCT. However, challenges in procuring matched unrelated donor (MUD) grafts due to high cost, ethnic disparity and time constraints have led to the exponential rise in the use of stem cells from human leukocyte antigen (HLA)-haploidentical family donors. Whilst HLA-haploidentical HSCT (hHSCT) performed in adult patients with acute leukaemia has produced outcomes similar to MUD transplants, experience in children is limited. Over the last 5 years, more data have emerged on hHSCT in the childhood ALL setting, allowing comparisons with matched donor transplants. The feasibility of hHSCT using adult family donors in childhood ALL may also address the ethical issues related to selection of minor siblings in matched sibling donor transplants. Here, we review hHSCT in paediatric recipients with ALL and highlight the emergence of hHSCT as a promising therapeutic option for patients lacking a suitable matched donor. Recent issues related to conditioning regimens, donor selection and graft-vs.-host disease prophylaxis are discussed. We also identify areas for future research to address transplant-related complications and improve post-transplant disease-free survival.
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Up to 40% of donor-recipient pairs in SCT have some degree of ABO incompatibility, which may cause severe complications. The aim of this study was to describe available options and survey current practices by means of a questionnaire circulated within the EBMT Pediatric Diseases Working Party investigators. Major ABO incompatibility (donor's RBCs have antigens missing on the recipient's cell surface, towards which the recipient has circulating isohemagglutinins) requires most frequently an intervention in case of bone marrow grafts, as immediate or delayed hemolysis, delayed erythropoiesis and pure red cell aplasia may occur. RBC depletion from the graft (82%), recipient plasma-exchange (14%) were the most common practices, according to the survey. Graft manipulation is rarely needed in mobilized peripheral blood grafts. In case of minor incompatible grafts (donor has isohemagglutinins directed against recipient RBC antigens), isohemagglutinin depletion from the graft by plasma reduction/centrifugation may be considered, but acute tolerability of minor incompatible grafts is rarely an issue. According to the survey, minor ABO incompatibility was either managed by means of plasma removal from the graft, especially when isohemagglutinin titer was above a certain threshold, or led to no intervention at all (41%). Advantages and disadvantages of each method are discussed.
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Sistema del Grupo Sanguíneo ABO , Incompatibilidad de Grupos Sanguíneos , Trasplante de Médula Ósea , Niño , Eritrocitos , Hemólisis , HumanosRESUMEN
A 2-week-old male newborn with a double inlet left ventricle developed a cardiac arrest following modified Blalock-Taussig anastomosis in pediatric intensive care unit. Probable causes of the arrest were hemodynamic instability and thrombosed shunt, which was later recanalized on extracorporeal membrane oxygenation therapy, which was successfully used with a pump flow lower than recommended in these patients-without the shunt clip, but without any complications.
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Procedimiento de Blalock-Taussing , Reanimación Cardiopulmonar , Oxigenación por Membrana Extracorpórea , Paro Cardíaco , Anastomosis Quirúrgica , Procedimiento de Blalock-Taussing/efectos adversos , Niño , Paro Cardíaco/etiología , Paro Cardíaco/terapia , Humanos , Recién Nacido , MasculinoRESUMEN
We present a case of a 16-year-old male with large pericardial effusion due to Hodgkin Lymphoma (HL). Shortly after drainage of pericardial effusion he developed a low cardiac output syndrome which had to be treated with extracorporeal membrane oxygenation (ECMO). This 9-day ECMO support helped the patient to recover his cardiac function, and thereafter a remission of his primary disease was successfully achieved with chemotherapy. It is a matter of discussion whether a large pericardial effusion with moderate symptoms in patients with HL should be evacuated or just observed since the effusion should ameliorate with chemotherapy. But based upon our experience in this case of hemodynamic instability due to a large effusion requiring evacuation, we propose that pericardiocentesis procedure should be performed with caution at a slow drainage rate of 0.5-1 ml/kg/hour with a maximum rate of 50 ml/hour, to help avoid the low cardiac output syndrome in patients with similar disease conditions.
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Taponamiento Cardíaco , Oxigenación por Membrana Extracorpórea , Enfermedad de Hodgkin , Adolescente , Gasto Cardíaco Bajo , Taponamiento Cardíaco/cirugía , Enfermedad de Hodgkin/complicaciones , Enfermedad de Hodgkin/terapia , Humanos , Masculino , PericardiocentesisRESUMEN
A five-month-old male with a hypoplastic left heart syndrome developed severe respiratory insufficiency due to influenza type B viral pneumonia following bidirectional Glenn. He was treated with extracorporeal membrane oxygenation and successfully weaned without neurological consequences.
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Procedimientos Quirúrgicos Cardíacos/efectos adversos , Oxigenación por Membrana Extracorpórea , Síndrome del Corazón Izquierdo Hipoplásico/complicaciones , Gripe Humana/complicaciones , Neumonía Viral/complicaciones , Puente Cardíaco Derecho , Humanos , Síndrome del Corazón Izquierdo Hipoplásico/fisiopatología , Lactante , Virus de la Influenza B , Gripe Humana/fisiopatología , Masculino , Neumonía Viral/fisiopatología , Síndrome de Dificultad RespiratoriaRESUMEN
Hematopoietic stem cell transplantation (HSCT) is currently the standard of care for many malignant and nonmalignant blood diseases. As several treatment-emerging acute toxicities are expected, optimal supportive measurements critically affect HSCT outcomes. The paucity of good clinical studies in supportive practices gives rise to the establishment of heterogeneous guidelines across the different centers, which hampers direct clinical comparison in multicentric studies. Aiming to harmonize the supportive care provided during the pediatric HSCT in Europe, the Pediatric Diseases Working Party (PDWP) of the European Society for Blood and Marrow Transplantation (EBMT) promoted dedicated workshops during the years 2017 and 2018. The present paper describes the resulting consensus on the management of sinusoidal obstructive syndrome, mucositis, enteral and parenteral nutrition, iron overload, and emesis during HSCT.
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Enfermedades Transmisibles , Trasplante de Células Madre Hematopoyéticas , Médula Ósea , Niño , Europa (Continente) , Humanos , InvestigaciónRESUMEN
An amendment to this paper has been published and can be accessed via a link at the top of the paper.
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A mini extracorporeal photopheresis (mini-ECP) "off line" technique has been developed for use in the treatment of small children and patients with apheresis contraindications. Until now various methods have been used for buffy coat separation from whole blood. In this report we describe a protocol for mini buffy coat preparation using the automated Sepax laboratory separator for "off line" ECP treatment in a low body weight child with graft-vs-host-disease. According to our results this alternative method has been proven feasible and tolerable.
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Capa Leucocitaria de la Sangre/citología , Enfermedad Injerto contra Huésped/terapia , Fotoféresis/métodos , Eliminación de Componentes Sanguíneos/métodos , Peso Corporal , Niño , Preescolar , Femenino , Humanos , Masculino , Resultado del TratamientoRESUMEN
Congenital hyperinsulinism (CHI) is a major cause of persistent hypoglycemia in the neonatal and early infancy periods. Althought the disease is relatively rare with incidence of about 1:25 000-50 000 live births, the importance of the disease should not be underestimated. Namely, prompt recognition and management of patients with CHI is essential, if permanent neurological impairment is to be avoided. CHI is caused by mutations in one of the 7 genes involved in the regulation of insulin secretion in pancreatic beta-cells. It is important to introduce specific medical therapy as soon as diagnosis is established. Severe, neonatal forms of CHI are often resistant to medications, thus they require surgical procedure. The preoperative genetic testing and scintigraphy are indicated to distinguish histological subtypes of the disease (focal vs. diffuse CHI). Patients with focal disease are usually cured after pancreatic resection, while diffuse disease has much worse prognosis. This manuscript offers novel insights into CHI and emphasizes the role of early diagnosis as crucial for succesful treatment that was recently enriched with novel options.
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Hiperinsulinismo Congénito , Hiperinsulinismo Congénito/diagnóstico , Hiperinsulinismo Congénito/genética , Hiperinsulinismo Congénito/terapia , Humanos , Recién NacidoRESUMEN
Up to now 36 cases of Marden Walker syndrome (MIM # 248700) have been reported. The underlying pathological mechanism has not yet been clearly established, but it is assumed that it is a developmental disorder of the central nervous system which is inherited in an autosomal recessive manner. Three main diagnostic criteria are blepharophimosis, congenital joint contractures and mask-like face, but numerous other anomalies have been described in these patients. We present a girl with clinical characteristics of Marden Walker syndrome, which has not yet been reported in our literature. Together with three major and many other clinical signs which confirm the diagnosis, our girl also has a vesicoureteral reflux and umbilical hernia, which have not yet been described in these patients.
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Anomalías Múltiples , Blefarofimosis , Preescolar , Contractura , Anomalías Craneofaciales , Femenino , Humanos , SíndromeRESUMEN
We report a 11-year-old girl and two 14-year-old boys with Cushing's syndrome due to primary pigmented nodular adrenocortical disease (PPNAD). In these patients, hypercortisolism is a consequence of autonomous cortisol secretion from adrenal glands and is ACTH-independent. Besides PPNAD, the girl had lentigines, spotty pigmentation on her bucal mucosa and lips and she also had schwannoma. One of the reported boys had prolactinoma. Considering this, those two patients fulfill the criteria for Carney complex which is a type of multiple endocrine neoplasia syndromes inherited in an autosomal dominant trait. The other boy had PPNAD but no other obvious signs of Carney complex were noticed. Family study didn't reveal any clinical or laboratory signs of Carney complex in our patients' first relatives. All of our patients underwent bilateral adrenalectomy (in one of the boys laparoscopic surgery was performed). Glucocorticoid and mineralocorticoid substitution has been started. Adrenal glands were macroscopically normal but pathohistological analysis confirmed the diagnosis of PPNAD.
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Enfermedades de la Corteza Suprarrenal/complicaciones , Síndrome de Cushing/etiología , Neoplasia Endocrina Múltiple/diagnóstico , Adolescente , Enfermedades de la Corteza Suprarrenal/diagnóstico , Enfermedades de la Corteza Suprarrenal/cirugía , Adrenalectomía , Niño , Síndrome de Cushing/diagnóstico , Femenino , Humanos , Hiperpigmentación/complicaciones , MasculinoRESUMEN
A 10-year-old girl with characteristic features of Keipert syndrome (broad terminal phalanges, especially of the thumb and hallux, sensorineural deafness, unusual facial features, large head circumference, maxillary hypoplasia, hoarse voice) and her mildly affected father (broad terminal phalanges, especially of the thumb and hallux, large head circumference, maxillary hypoplasia, and hoarse voice) are presented. The girl is the first reported female with this rare syndrome to date, and the fact that she probably inherited the disease from her father suggests an autosomal dominant pattern of inheritance.