RESUMEN
BACKGROUND: Chronic Myeloid Leukemia (CML) is a rare myeloproliferative disease in childhood. Treatment in CML includes Tyrosine Kinase Inhibitors (TKI's), which inhibit the cytoplasmic kinase BCR/ABL. Tyrosine kinases play a key role in the secretion of growth hormone and insulin-like growth factor1 (IGF-1). OBJECTIVE: The aim of this systematic review was to study the effect of TKIs on the growth of children and adolescents with CML. METHODS: English-language publications were searched in the PubMed/Cochrane library/Google Scholar databases (2002-2023), and retrieved studies were assessed according to PRISMA-Statement and Newcastle- Ottawa-scale. RESULTS: The search strategy yielded 1066 articles. After applying the inclusion/exclusion criteria, 941 were excluded based on title screening and 111 on abstract review. The systematic review included 14 articles (11 retrospective observational studies/3 clinical trials). Twelve studies reported data on the prevalence of growth disorders after the administration of 1st generation TKIs (imatinib). Two studies reported a negative effect of 2nd generation TKIs (dasatinib/nilotinib) on physical growth. Four studies recorded a decrease in height z-score after treatment compared to baseline. Two 1st-generation TKIs studies reported data on children's final height; one reported restoration of final height to normal after the onset of puberty, despite initial slowing, and the final height was lower than mid-parental target height. Serum IGF-1 levels were reported in 2 studies to be within normal range, while in 3 studies, a significant decrease was documented. Considerable study heterogeneity was observed related to dosage/duration of treatment/disease phase/stage of puberty/ethnicity. CONCLUSION: A negative effect of TKIs on the growth and final height of children was noted.
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Cancer as a whole, but especially childhood cancer, creates a number of psychological, social, and family problems as well as practical and financial issues, which every parent is called upon to solve. This study focuses on childhood cancer and aims at a thorough analysis of the physical/organic, psychological, and social problems associated with the parents and relatives of a child with cancer. The special element in pediatric neoplasms is not only the vulnerable population target group, but also the set of secondary effects it has on the environment of the sick child. The research was conducted on a sample of 133 families of children with cancer, and the results were displayed after statistical processing and data analysis with R statistical software. The results of the study confirm with statistically significant data the effect of childhood cancer on the physical, mental, and social health and behavior of the parent. Thus, 53.8% of the respondents stated 5 and above on the 7-point Likert scale for fatigue issues, 55.6% for sleep disorders, 78.1% for stress, and 82.7% for fear. The key findings are characterized by high specificity as it is a unique study that reveals particular aspects of the Greek parent's behavior, mind, and body during the period of their child's illness. Conclusion: The effects of childhood illnesses, particularly when they are severe, such as neoplasms, present a looming threat, ushering in a multitude of adverse alterations in the daily lives of the affected child's family. What is Known - What is New: ⢠We know the effects that a childhood illness brings not only to the sick child, but also to the entire family circle. The new element in the present research is that these data reflect the situation in Greece, for which the research data in this area is quite limited. Our research is one of the few studies that demonstrate with statistical data the change in the psychosomatic health of the parent who has a child with cancer.
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Neoplasias , Humanos , Niño , Grecia , Padres/psicologíaRESUMEN
BACKGROUND: Adolescence is accompanied by a variety of changes in young breast development, which greatly affects the adolescent's psychology and socialization. SUMMARY: PubMed, EMBASE, and the Cochrane Library were searched for studies relative to epidemiology, clinical characteristics, diagnosis, and management of all breast disorders in adolescence and their consequences. Development disorders are breast asymmetry, breast atrophy, breast hypoplasia, hypomastia, juvenile breast hypertrophy, and tuberous breast. Breast congenital abnormalities include athelia, amastia, accessory breast tissue, polymastia, polythelia, and congenital disorders of nipples. Breast infections are commonly caused from Gram-positive coccus rather than Gram-negative bacteria. Breast abscess occurs when breast infections are not promptly treated. Nipple discharge is caused by a variety of conditions and should be managed carefully. Fibrocystic changes, cysts, and fibroadenomas are the most common benign masses in adolescence. Primary, secondary, or metastatic breast cancer is extremely rare in adolescence. However, clinicians should include breast cancer in the differential diagnosis of a breast mass in adolescence. KEY MESSAGES: Clinicians should be aware of all breast disorders that may occur in adolescence. Early diagnosis and treatment will result in the reassurance of adolescents and their families without any detrimental effect on their psychology, sexual behavior, and socialization. Adolescents with breast disorders may require a multidisciplinary approach by a pediatrician, a gynecologist specializing in pediatric-adolescent gynecology, a plastic surgeon, and a psychologist for the best management of breast disorders.
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Astrocitoma/patología , Neoplasias Encefálicas/patología , Neoplasias Primarias Secundarias/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Astrocitoma/cirugía , Neoplasias Encefálicas/cirugía , Preescolar , Humanos , Imagen por Resonancia Magnética , Masculino , Neoplasias Primarias Secundarias/terapia , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapiaRESUMEN
Loefler endocarditis is a potential fatal adverse event of hypereosinophilic syndrome. We report a case of a 5-year-old girl diagnosed with peripheral hypereosinophilia refractory to corticosteroid therapy who developed eosinophilia-related endocarditis. Echocardiography revealed infiltration of the left ventricular free wall and the posterior mitral leaflet causing moderate mitral regurgitation. Genetic tests failed to recognize FIPiLi-PDGRFA genotype; however imatinib, a tyrosine kinase inhibitor was initiated. After a 4-week period of treatment there was a complete resolution of eosinophilia and a complete recovery of cardiac manifestation. This case highlights the introduction of imatinib for the treatment of hypereosinophilic syndrome refractory to corticosteroid therapy even in the absence of FIPiLi-PDGRFA genotype in pediatric patients.
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Antineoplásicos/administración & dosificación , Benzamidas/administración & dosificación , Síndrome Hipereosinofílico , Piperazinas/administración & dosificación , Pirimidinas/administración & dosificación , Preescolar , Electrocardiografía , Femenino , Humanos , Síndrome Hipereosinofílico/complicaciones , Síndrome Hipereosinofílico/tratamiento farmacológico , Síndrome Hipereosinofílico/genética , Síndrome Hipereosinofílico/fisiopatología , Mesilato de ImatinibAsunto(s)
Isquemia Encefálica/complicaciones , Infarto Cerebral/complicaciones , Infarto Cerebral/etiología , Neurofibromatosis 1/complicaciones , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Isquemia Encefálica/diagnóstico , Angiografía Cerebral , Preescolar , Humanos , Angiografía por Resonancia Magnética , Imagen por Resonancia Magnética , Masculino , Neurofibromatosis 1/diagnósticoRESUMEN
BACKGROUND AND OBJECTIVE: Carcinoid tumors of the appendix are rare in childhood and usually have a benign clinical course. Their incidence in appendectomy specimens ranges from 0.1% to 0.9%. The aim of the study was to report the frequency, clinical presentation, tumor characteristics, and outcome of children with appendiceal carcinoid treated in a pediatric oncology department. PATIENTS AND METHODS: All of the cases referred during a 19-year period (1990-2008) were studied retrospectively. Demographics, clinical presentation, tumor characteristics, and follow-up results were recorded. RESULTS: Among 839 admissions, 19 patients (9 boys) with appendiceal carcinoid were identified during the study period. Their median age was 10.5 years (range 4.5-13.2 years). In all of the cases, diagnosis was established after appendectomy. The mean tumor diameter was 4.55 (± 3.45) mm (range 1-15 mm). Concomitant appendicitis was diagnosed in 12 patients. In 18 children tumor size was ≤ 10 mm and did not infiltrate surrounding tissues. In 1 patient the size was 15 mm and a microscopic rupture of the appendix with infiltration of the surrounding fat was present. All of the tumors were located at the tip of the appendix and were of the classic histological type. Staging and follow-up consisted of abdominal ultrasound, chest and abdominal computed tomography scans, Tc bone scan, urine 5-hydroxylindoloacetic acid levels, and 111In octreotide scan. No patient had metastases requiring further therapeutic interventions. No relapses or other neoplasms occurred during a median follow-up period of 45 months (range 6-118 months). CONCLUSIONS: Carcinoid tumors of the appendix in children are rare. Long-term follow-up revealed that a good prognosis is possible provided they are diagnosed and surgically removed at an early stage.
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Apendicectomía , Neoplasias del Apéndice , Apendicitis/complicaciones , Tumor Carcinoide , Adolescente , Neoplasias del Apéndice/complicaciones , Neoplasias del Apéndice/patología , Neoplasias del Apéndice/cirugía , Apendicitis/patología , Apendicitis/cirugía , Apéndice/patología , Apéndice/cirugía , Tumor Carcinoide/complicaciones , Tumor Carcinoide/patología , Tumor Carcinoide/cirugía , Niño , Femenino , Humanos , Incidencia , Masculino , Estudios RetrospectivosRESUMEN
Neuroblastoma is the most common extracranial solid tumor in children. Survival rates have improved due to advances in treatment with aggressive chemotherapy and autologous bone marrow transplantation. Usual sites of recurrence include the site of primary tumor, residual gross disease, bone, bone narrow, liver, and lungs. The authors describe a 16-month-old boy with stage IV extracerebral primary neuroblastoma who died because of an isolated central nervous system (CNS) relapse. The CNS is a rare site of relapse that is, however, increasingly diagnosed due to prolonged survival. Criteria to identify patients at increased risk of CNS relapse are urgently needed. High-risk patients should be followed-up with brain and spine magnetic resonance imaging (MRI) for timely detection of metastases and appropriate management.
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Neoplasias del Sistema Nervioso Central/diagnóstico , Neuroblastoma/patología , Sistema Nervioso Central/patología , Resultado Fatal , Humanos , Lactante , Imagen por Resonancia Magnética , Masculino , Metástasis de la Neoplasia/diagnóstico , Recurrencia , RiesgoAsunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Coagulantes/uso terapéutico , Coagulación Intravascular Diseminada/tratamiento farmacológico , Factor VIIa/uso terapéutico , Hemorragia/tratamiento farmacológico , Neuroblastoma/tratamiento farmacológico , Coagulación Intravascular Diseminada/inducido químicamente , Hemorragia/inducido químicamente , Humanos , Lactante , Masculino , Proteínas Recombinantes/uso terapéutico , Factores de Tiempo , Resultado del TratamientoRESUMEN
OBJECTIVE: Osteopenia/osteoporosis of multi-factorial pathogenetic mechanism is reported to be a significant cause of morbidity in adult patients with beta-thalassaemia major. Even in young patients, decreased Bone Mineral Density (BMD) values are a consistent finding in the literature. This study was performed in order to assess BMD in children and young adults with beta-thalassaemia major, regularly transfused and sufficiently chelated, along with auxological, clinical and laboratory parameters. DESIGN: Thirty-five young thalassaemic patients (19 F, 16 M, aged 5-20 yr) were studied. Lumbar BMD was assessed by dual X-ray absorptiometry (DXA) and Z-scores were calculated according to bone density values using age- and sex-matched normal population. None of the patients presented with clinical or laboratory signs of endocrinopathy and none was receiving hormonal replacement therapy. RESULTS: All BMD Z-scores were within normal range, with a mean Z-score of 0.42 for girls and -0.41 for boys (statistically significant gender difference, p=0.018). When correlated with age, a decline in Z-scores was observed, indicating a delay in bone mass acquisition with advancing age in the thalassaemic group compared to controls. CONCLUSIONS: Optimal conventional treatment prevents the manifestation of osteopenia/osteoporosis during the first two decades of life in patients with beta-thalassaemia major. However, close surveillance with regular screening, preventive intervention and early management of possible endocrine complications are essential in order to secure normal bone health during adulthood and improve quality of life in the thalassaemic population.
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Densidad Ósea , Talasemia beta/fisiopatología , Talasemia beta/terapia , Absorciometría de Fotón , Adolescente , Adulto , Transfusión Sanguínea , Estatura , Quelantes/uso terapéutico , Niño , Preescolar , Deferiprona , Deferoxamina/uso terapéutico , Femenino , Humanos , Proteína 3 de Unión a Factor de Crecimiento Similar a la Insulina/sangre , Factor I del Crecimiento Similar a la Insulina/análisis , Vértebras Lumbares , Masculino , Piridonas/uso terapéuticoRESUMEN
Hemophilic pseudotumor is an uncommon complication seen in approximately 1-2% of patients with severe hemophilia. Hemophilic pseudotumors are distinguished into two subdivisions based on location, proximal or distal. Plain x-rays and CT are useful in diagnosis, but MR imaging is the diagnostic test of choice because of its sensitivity to the various blood products. The choice of therapy depends on many parameters, such as the size of the tumor, the age of the patient, and the relation with underlying organs. In most cases of asymptomatic hemophilic pseudotumor, conservative treatment with administration of missing factor as well as immobilization is recommended. The authors describe a 13-year-old boy with severe hemophilia A, who presented with a tibial pseudotumor a few months after an injury. He was conservatively treated for a long period, with daily administration of recombinant factor VIII. His clinical condition improved shortly after therapy induction, but radiological improvement has been moderate. Case history, imaging findings, and therapeutic options are discussed.
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Neoplasias Óseas/diagnóstico por imagen , Hemofilia A/diagnóstico por imagen , Tibia/diagnóstico por imagen , Adolescente , Neoplasias Óseas/tratamiento farmacológico , Neoplasias Óseas/etiología , Diagnóstico Diferencial , Factor VIII/administración & dosificación , Hemofilia A/complicaciones , Hemofilia A/tratamiento farmacológico , Humanos , Imagen por Resonancia Magnética , Masculino , Factores de Tiempo , Tomografía Computarizada por Rayos XAsunto(s)
Terapia por Quelación/métodos , Talasemia beta/tratamiento farmacológico , Adolescente , Adulto , Terapia por Quelación/efectos adversos , Deferiprona , Deferoxamina/administración & dosificación , Deferoxamina/efectos adversos , Quimioterapia Combinada , Ferritinas/sangre , Homocigoto , Humanos , Sobrecarga de Hierro/tratamiento farmacológico , Piridonas/administración & dosificación , Piridonas/efectos adversos , Talasemia beta/terapiaRESUMEN
Hemoglobinopathies are very common in Greece, the incidence of beta-thalassemia trait being 8% and that of sickle cell trait ranging from 1 to 32% in various districts. In Greek populations, sickle cell disease (SCD) is mainly represented by S-beta thalassemia.