How to manage patients with germline variants: Recommendations from the Nordic working group on germline predisposition for myeloid neoplasms.

Increasing recognition of germline variants in patients with hematological malignancies prompted us to provide -specific recommendations for diagnosis, surveillance, and treatment. Causative germline variants in the predispose to the development of myeloid neoplasms (MNs), especially myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML). Almost 3%-5% of all patients with MDS or AML carry a pathogenic or likely pathogenic germline variant, while half of them acquire a somatic second hit in the other allele.

Cardiac Structure and Function in 8- to 12-Year-Old Children Following In-Utero Exposure to Preeclampsia (FINNCARE Study).

Background: We evaluated how elevated blood pressure in children exposed to preeclampsia (PE) impacted on their cardiac structure and function, as well as relations with maternal, gestational, and perinatal factors and child body size and composition.

Methods And Results: A total of 182 PE (46 early-onset preeclampsia) and 85 unexposed (non-PE) children were examined in the FINNCARE study 8 to 12 years after the index pregnancy with echocardiography; office, central, and 24-hour ambulatory blood pressures; and body anthropometrics and composition. PE children had lower right ventricular basal sphericity index (mean difference, -0.

Mode of delivery of Finnish dichorionic and monochorionic-diamniotic twins: A retrospective observational study including a risk score for intrapartum cesarean birth.

Background And Aims: Trial of labor is considered safe also among twins, yet nearly 50% are born via cesarean section in Finland. While planned cesarean births have declined among twins, intrapartum cesarean deliveries have risen, postulating evaluation of criteria for trial of labor. The objective of this study was to create an outline of the mode of delivery of dichorionic and monochorionic-diamniotic Finnish twins.

Changes in emotional problems, hyperactivity and conduct problems in moderate to late preterm children and adolescents born between 1958 and 2002 in the United Kingdom.

Background: Preterm birth is a risk factor for the development of emotional and behavioural problems in childhood and adolescence. Given the substantial improvements in neonatal care across decades, it has been expected that the difference in emotional problems, hyperactivity, and conduct problems between moderate to late preterm (MLPT) and full term (FT) children and adolescents have declined in recent years.

Methods: Data from four UK population-based studies were used: The National Child Development Study (NCDS; 1958), the British Cohort Study (BCS70; 1970), the Avon Longitudinal Study of Parents and Children (ALSPAC, 1991-1992) and the Millennium Cohort Study (MCS; 2000-2002).

Chronic Disease Burden After Congenital Heart Surgery: A 47-Year Population-Based Study With 99% Follow-Up.

Background Postoperative morbidity is an increasingly important outcome measure of patients who have undergone congenital heart surgery (CHS). We examined late postoperative morbidity after CHS on the basis of patients' government-issued medical special reimbursement rights. Methods and Results Between 1953 and 2009, 10 635 patients underwent CHS at <15 years of age in Finland.

Outcomes of surgical management of familial intrahepatic cholestasis 1 and bile salt export protein deficiencies.

Progressive familial intrahepatic cholestasis (PFIC) with normal circulating gamma-glutamyl transpeptidase levels can result from mutations in the gene (encoding familial intrahepatic cholestasis 1 [FIC1] deficiency) or the gene (bile salt export protein [BSEP] deficiency). We investigated the outcomes of partial external biliary diversion, ileal exclusion, and liver transplantation in these two conditions. We conducted a retrospective multicenter study of 42 patients with FIC1 deficiency (FIC1 patients) and 60 patients with BSEP deficiency (BSEP patients) who had undergone one or more surgical procedures (57 diversions, 6 exclusions, and 57 transplants).

Outcomes of renal replacement therapy in boys with prune belly syndrome: findings from the ESPN/ERA-EDTA Registry.

Background: As outcome data for prune belly syndrome (PBS) complicated by end-stage renal disease are scarce, we analyzed characteristics and outcomes of children with PBS using the European Society for Pediatric Nephrology/European Renal Association-European Dialysis and Transplant Association (ESPN/ERA-EDTA) Registry data.

Methods: Data were available for 88 male PBS patients aged <20 years who started renal replacement therapy (RRT) between 1990 and 2013 in 35 European countries. Patient characteristics, survival, and transplantation outcomes were compared with those of male patients requiring RRT due to congenital obstructive uropathy (COU) and renal hypoplasia or dysplasia (RHD).

Survival and clinical outcomes of children starting renal replacement therapy in the neonatal period.

End-stage renal disease requiring renal replacement therapy (RRT) during the neonatal period is a very rare condition, and little information is available regarding long-term RRT and outcomes. To gain more information, we performed a collaborative study on patient characteristics and treatment outcomes in children who started RRT as neonates during their first month of life between 2000 and 2011 who were prospectively registered in the ESPN/ERA-EDTA, the IPPN (since 2007), the Japanese registry, or the Australian and New Zealand Dialysis and Transplant (ANZDATA) registry. During the first month of life, 264 patients from 32 countries started RRT and were followed for a median of 29 months (interquartile range 11-60 months).

Modern outcomes of oesophageal atresia: single centre experience over the last twenty years.

Aim Of The Study: The aim of this study was assessment of the contemporary outcomes of oesophageal atresia (OA) in a national paediatric surgical centre.

Patients And Methods: A review of the hospital records of all patients who underwent repair of oesophageal atresia (OA) in our institute between 1991 and 2011 was performed.

Results: The study included 130 consecutive infants with OA: types A (n=4, 3%), B (n=2, 2%), C (n=110, 85%), D (n=5, 4%), and E (n=9, 7%).

Endoscopic follow-up in esophageal atresia-for how long is it necessary?

Purpose: The aim of this study was to find out how long it is necessary to follow-up esophageal atresia patients endoscopically to prevent irreversible mucosal changes.

Methods: Seventy-four of 79 long-term survivors with esophageal atresia and primary anastomosis underwent a total of 322 esophagogastroduodenoscopies during a follow-up period of 0.5 to 19 years (mean, 10.

The pharmacodynamics of mivacurium preceded by atracurium or cisatracurium in children.

Unlabelled: We evaluated whether mivacurium maintains its short duration of effect when preceded by atracurium or cisatracurium in 45 children during propofol/alfentanil/N2O/O2 anesthesia. Neuromuscular response was recorded by using an adductor pollicis electromyogram (EMG). Children were randomized to receive two doses of atracurium (350 micrograms/kg and 70 micrograms/kg in Group AM), cisatracurium (64 micrograms/kg and 10 micrograms/kg in Group CM), or mivacurium (200 micrograms/kg and 100 micrograms/kg in Group MM), followed by a final dose of mivacurium 100 micrograms/kg.

Can final EMG baseline be used as a reference to calculate neuromuscular recovery?

Background: Investigations recording recovery times of muscle relaxants have used initial or final baseline of a neuromuscular trace, or both, as a reference for data analysis. We evaluated the use of final baseline of EMG traces as a reliable reference to calculate recovery times.

Methods: We analyzed EMG traces from 82 children who had full spontaneous neuromuscular recovery following a single dose of mivacurium.

Pipecuronium revisited: dose-response and maintenance requirement in infants, children, and adults.

Study Objective: To compare dose-response relationship and maintenance requirement of pipecuronium in anesthetized infants, children, and adults.

Design: Prospective, consecutive sample trial.

Setting: Operating room at a university hospital.

Sevoflurane-nitrous oxide or halothane-nitrous oxide for paediatric bronchoscopy and gastroscopy.

We have studied 120 infants and children, in three age groups (3-11 months, 1-5 yr and 6-15 yr), to compare anaesthesia with sevoflurane or halothane for bronchoscopy or gastroscopy, or both. Premedication or i.v.

Potency of doxacurium in infants, children, and adolescents during N2O-O2-alfentanil anesthesia.

Study Objective: To evaluate neuromuscular potency of doxacurium during balanced anesthesia in pediatric patients.

Design: Prospective, consecutive sample trial.

Setting: Operating room at a university hospital.

Time course of potentiation of mivacurium by halothane and isoflurane in children.

We studied 40 children, aged 1-15 yr, to analyse the time course of potentiation of mivacurium produced by halothane and isoflurane. A steady infusion requirement of mivacurium to maintain 90% neuromuscular block was established during thiopentone-alfentanil-nitrous oxide-oxygen anaesthesia. Patients were then allocated randomly to receive 1 MAC end-tidal concentration of either halothane (group Hal) or isoflurane (group Iso) while neuromuscular block was maintained at 90%.

Effect of succinylcholine on subsequently administered mivacurium in children.

The interaction between mivacurium and succinylcholine when mivacurium was administered during the early recovery from succinylcholine block was studied in 30 children 2-12 years of age anaesthetized with propofolalfentanil-N2O-O2. Neuromuscular response was monitored by adductor pollicis EMG. Fifteen patients received 200 micrograms.

Dose-response and time-course of effect of rocuronium bromide in paediatric patients.

We determined dose-response curves and spontaneous recovery of neuromuscular function following a single ED95 dose of rocuronium in 10 infants (1-11 months old), 20 children (2-12 years old) and 20 adults (20-40 years old) during nitrous oxide-oxygen-thiopentone-alfentanil anaesthesia. Neuromuscular block was recorded as adductor pollicis EMG following a supramaximal train-of-four ulnar nerve stimuli at 10 s intervals. ED95 values were significantly less for infants than for children or adults (255 +/- 62, 402 +/- 68 and 350 +/- 78 micrograms kg-1, respectively, P = 0.

Infantile-onset spinocerebellar ataxia: MR and CT findings.

Purpose: To report the MR and CT findings in a hereditary disease, infantile-onset spinocerebellar ataxia (IOSCA).

Methods: We studied the brains of 17 patients with infantile-onset spinocerebellar ataxia with CT and/or MR to determine the presence of cerebellar and brain stem atrophy and parenchymal lesions.

Results: Cerebellar cortical atrophy was seen in 13 patients.

Pharmacodynamic effects of 51W89, an isomer of atracurium, in children during halothane anaesthesia.

51W89 is one of the 10 stereoisomers of atracurium with less propensity to release histamine than atracurium. We evaluated dose-response data and neuromuscular effects of 2 x ED95 dose and maintenance doses of 51W89 during halothane anaesthesia in 68 children, 2-12 yr old. Neuromuscular function was monitored by evoked adductor pollicis EMG.

Pharmacodynamics of mivacurium in infants.

A computerized infusion system was used to determine mivacurium infusion requirements to maintain 95% and 50% neuromuscular block in 15 infants less than 1 yr of age. Neuromuscular block was measured by adductor pollicis EMG and anaesthesia maintained with 66% nitrous oxide in oxygen and alfentanil 50-100 micrograms kg-1 h-1. Neuromuscular block was produced by repeated bolus doses of mivacurium 0.

Frequency of train-of-four stimulation influences neuromuscular response.

We have compared the effects of two different frequencies of train-of-four stimulation of the ulnar nerve (2-Hz stimulation once every 10 or 20 s) on onset time and potency of atracurium, vecuronium and mivacurium during balanced anaesthesia. The adductor pollicis EMG was recorded simultaneously in both hands of 24 children aged 2-12 yr. After administration of an ED50 dose of each blocker, onset times were mean 21 (SEM 10) s shorter (P < 0.

Synergism between atracurium and vecuronium in children.

In 30 children under balanced anaesthesia, we have determined dose-response curves and maintenance requirement of three dose ratio combinations of atracurium and vecuronium (10:1, 4:1 or 1.6:1 on a microgram:microgram basis). Neuromuscular block was monitored by adductor pollicis EMG.