Among patients with a monoallelic ATP-sensitive potassium (KATP) channel mutation, 50.0% showed gradual remission during follow up, indicating that the octreotide treatment may be a feasible alternative to surgery, especially for patients with monoallelic KATP-channel mutations. Transient elevation of liver enzymes occurred in 20.0% of patients, while asymptomatic gallbladder pathology occurred in one patient. The growth rates of these patients were normal (height standard deviation score was 0.3 ± 1.5 at the final follow-up).

Octreotide was a well-tolerated, effective therapy for most children with diazoxide-unresponsive CHI.

Octreotide was a well-tolerated, effective therapy for most children with diazoxide-unresponsive CHI.

Dexmedetomidine inhibits the inflammatory response associated with cardiopulmonary bypass (CPB) and protects neural function. However, the mechanism of dexmedetomidine’s anti-inflammatory pathway is unclear.

To investigate the effect of dexmedetomidine on the cognitive level and expression of inflammatory factors in children with congenital heart disease undergoing intraoperative CPB.

Ninety children with congenital heart disease were recruited and randomly divided into 3 groups of 30 children in each. In Group 1, a 1.0 µg·kg

·h

intravenous bolus of dexmedetomidine was administered 10 minutes after induction of anesthesia, followed by a 0.2 µg·kg

·h

infusion until the surgical incision. In Group 2, a 0.5 µg/kg intravenous bolus of dexmedetomidine was administered 10 minutes after induction of anesthesia, followed by a 0.1 µg·kg

·h

infusion until the surgical incision. The control group was given physiological saline using the same method as in Groups 1 and 2. The serum levels of nuclear factor- NSE levels indicated that the nervous system was damaged. Administration of dexmedetomidine to children with congenital heart disease undergoing intraoperative CPB can inhibit the inflammatory response and may ameliorate the neurodevelopmental damage caused by CPB.

The increases in NF-κB, TNF-α, and IL-6 levels indicated aggravation of the inflammatory reaction and the increase S-100β protein and NSE levels indicated that the nervous system was damaged. Administration of dexmedetomidine to children with congenital heart disease undergoing intraoperative CPB can inhibit the inflammatory response and may ameliorate the neurodevelopmental damage caused by CPB.

There is a high incidence of iron deficiency in children worldwide. Notably, however, while iron deficiency is the most common cause of anemia, little is known about the prevalence and different types of iron deficiency in neuroblastoma patients.

The aim of the present study was to investigate the prevalence of iron deficiency in patients newly diagnosed with neuroblastoma.

A total of 195 newly diagnosed neuroblastoma patients from November 2015 to January 2018 were analyzed retrospectively. The survival analysis was estimated by the Kaplan-Meier method.

Of the 195 neuroblastoma patients included in the study, 121 (62.1%) had iron deficiency, 55 (28.2%) had absolute iron deficiency, and 66 (33.9%) had functional iron deficiency. Being aged ≥ 18 months, tumor originating in the abdomen, International Neuroblastoma Risk Group Staging System M, high-risk neuroblastoma, lactate dehydrogenase ≥ 1500 U/L, neuron-specific enolase ≥ 100 U/L, unfavorable histologic category,

amplification, chromosome 1p loss, and bone marrow metastasis were associated with significantly higher rates of functional iron deficiency (

0.05).

Functional iron deficiency at the time of initial neuroblastoma diagnosis predicted lower event-free survival. Long-term effects of iron supplementation in neuroblastoma patients with different types of iron deficiency need to be further studied.

Functional iron deficiency at the time of initial neuroblastoma diagnosis predicted lower event-free survival. Long-term effects of iron supplementation in neuroblastoma patients with different types of iron deficiency need to be further studied.

Pathogenic variants in the

gene are associated with aggressive dilated cardiomyopathy (DCM). Recently,

was found to be associated with left ventricular non-compaction cardiomyopathy (LVNC). Thus far, only five families with LVNC have been reported to carry variants in

. It remains unknown whether the variants in

associated with DCM can also cause LVNC.

To elucidate the causative

variant in two unrelated patients with both LVNC and DCM, and to identify the clinical characteristics associated with variants in

.

Trio whole-exome sequencing (WES) was performed. Variants were filtered and classified in accordance with the guidelines of the American College of Medical Genetics and Genomics (ACMG).

We identified two distinct

variants in

(one per patient) in these two patients with LVNC. Both variants have been reported in patients with DCM, without the LVNC phenotype. Patient 1 was an 11-year-old girl who had DCM, LVNC, and heart failure; the ratio of noncompacted-to-compacted myocardium was 2.71. A

heterozygous variant c.1907G>A (p.Arg636His) in exon 9 was identified in this patient. Patient 2 was a 13-year-old boy who had clinical phenotypes identical to those of Patient 1; the ratio of noncompacted-to-compacted myocardium was 3.21 in this patient. WES revealed a

heterozygous variant c.1909A>G (p.Ser637Gly) in exon 9. Both variants were previously characterized as pathogenic, and our study classified them as pathogenic variants based on the ACMG guidelines.

We found that two patients with LVNC had variants in

. Our results extended the clinical spectrum of the two

variants and illustrated that the same variant in

can cause DCM, with or without the LVNC phenotype.

We found that two patients with LVNC had variants in RBM20. Our results extended the clinical spectrum of the two RBM20 variants and illustrated that the same variant in RBM20 can cause DCM, with or without the LVNC phenotype.

In low resource countries, there has been scarcity of research on the risk factors associated with neutropenic enterocolitis, a serious complication that commonly develops during treatment of cancer patients.

To identify the pattern of intestinal complications in pediatric leukemia patients treated with intensive chemotherapy, including those with neutropenic enterocolitis; to assess the outcome; and to evaluate the risk factors associated with the mortality in these patients.

During the period from June 2015 to December 2016, a prospective study was carried out on pediatric patients diagnosed with acute leukemia who received induction/or re-induction phases of chemotherapy at South Egypt Cancer Institute. Patients with documented episodes of intestinal complications were included in the study. Recovery or death from an episode of intestinal complication was utilized as the primary outcome measure for the study. ALK inhibition Using univariable and multivariable methods, potential risk factors associated with mortality were delineated by logistic regression analysis, both for the entire intestinal complications episodes as a whole and for those episodes of neutropenic enterocolitis only.