Makale Arama

Macroglossia, prenatal or postnatal overgrowth, macrosomia, macroglossia, and abdominal wall defects (omphalocele, umbilical hernia, or diastasis recti) permit early recognition of BeckwithWiedemann syndrome. Complications include neonatal hypoglycemia and an increased risk for Wilms tumor, adrenal cortical carcinoma, hepatoblastoma, rhabdomyosarcoma, and neuroblastoma, among others. The frequency of hypoglycemia in this population is between 30% and 50%. The cause of hypoglycemia is unclear but supports a hyperinsulinemia as the major factor. The majority of infants with hypoglycemia will be asymptomatic and have resolution of the hypoglycemia within the first 3 days of life. Less than 5% will have hypoglycemia beyond the neonatal period. As conclusion, in patients with BWS levels of insulin and blood glucose must be monitorized beginning from first hours of life. Hypoglycemia may be prolonged in presence of hyperinsulinemia as like this present case.

Aim: In this study, we discuss the efficacy and safety of autologous hematopoietic stem cell transplantation
(AHSCT) for high-risk pediatric solid-tumor patients based on 1 2 years of experience.
Patients and Methods: The data of patients aged < 18 years with pediatric malignancies who underwent
AHSCT between January 2009 and July 2021 at the Pediatric Bone Marrow Transplant Unit were evaluated
retrospectively.
Results: Fifty-one patients (24 girls and 27 boys; median age, 7.8 years; range: 0.5–18 years) were
enrolled in the study; 20, 15, 8, 4, 2, and 2 patients had diagnoses of neuroblastoma, Ewing’s sarcoma,
Hodgkin’s lymphoma, non-Hodgkin’s lymphoma, germ cell tumor, and soft tissue sarcoma, respectively.
The median neutrophil and platelet engraftment times were 11 (8–45) and 16 (4–62) days, respectively,
and the median hospital stay was 38 (17–67) days. The median follow-up time was 2.83 (0.12–10.81)
years and the overall survival (OS) rate was 51.3 ± 10.3% for all patients; the median follow-up times
and survival rates for the Ewing’s sarcoma and neuroblastoma cases were 2.63 (0.12–10.55) years and
64.2 ± 14.9%, and 2.81 (0.31–7.91) years and 42.8 ± 12%, respectively. All four patients who received
the conditioning regimen of carboplatin, etoposide, and melphalan (CEM) died; 6 of 16 neuroblastoma
patients who received the busulfan and melphalan (Bu/Mel) regimen as a conditioning regimen died: the
median follow-up period of the Bu/Mel neuroblastoma patients was 3.08 (0.32–7.91) years and the OS
rate was 55.1 ± 13.8%.
Conclusion: Although the number of patients in this study was limited, AHSCT resulted in better survival
for Ewing’s sarcoma and neuroblastoma cases than reported for those who did not undergo AHSCT in
our clinic.