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1.
Dev Sci ; : e13520, 2024 Apr 25.
Article in English | MEDLINE | ID: mdl-38664600

ABSTRACT

Across development, as children acquire a deeper understanding of their environment, they explore less and take advantage, or "exploit," what they already know. Here, we test whether children also enforce exploration-oriented search behaviors onto others. Specifically, we ask whether children are more likely to encourage a search agent to explore versus exploit their environment, and whether this pattern varies across childhood (between 3 and 6 years). We also ask whether this pattern differs between children and adults, and generalizes across two different sociocultural contexts-Turkey and the United States-that differ on dimensions that might relate to children's decisions about exploration (e.g., curiosity-focused educational practices, attitudes toward uncertainty avoidance). Participants (N = 358) watched an agent search for rewards and were asked at various points whether the agent should "stay" (exploit) in their current location, or "go" (explore) to a new location. At all points in the experiment, children enforced exploration significantly more often than adults. Early in the agent's search, children in the US enforced exploration more often than children in Turkey; later in the search, younger children (from both sociocultural contexts) were more likely to continue enforcing exploration compared to older children. These findings highlight that children are not only highly exploratory themselves, but also enforce exploration onto others-underscoring the central role that exploration plays in driving early cognitive development across diverse sociocultural contexts. RESEARCH HIGHLIGHTS: The current study examined developmental and cross-cultural differences in children and adults' enforcement of explore-exploit search strategies. Children in the US and Turkey enforced exploration more than adults, who enforced exploitation more often; results were generally consistent across cultures with small differences. Mirroring developmental changes in children's own search behavior; the tendency to enforce exploration decreased between 3- to 6-years of age. Findings underscore the central role of an "exploration mindset" in children's early decision-making-even when exploration has no direct benefits to the child themselves.

2.
Infant Behav Dev ; 74: 101927, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38428279

ABSTRACT

Infants actively initiate social interactions aiming to elicit different types of responses from other people. This study aimed to document a variety of communicative interactions initiated by 18-month-old Turkish infants from diverse SES (N = 43) with their caregivers in their natural home settings. The infant-initiated interactions such as use of deictic gestures (e.g., pointing, holdouts), action demonstrations, vocalizations, and non-specific play actions were coded from video recordings and classified into two categories as need-based and non-need-based. Need-based interactions were further classified as a) biological (e.g., feeding); b) socio-emotional (e.g., cuddling), and non-need-based interactions (i.e., communicative intentions) were coded as a) expressive, b) requestive; c) information/help-seeking; d) information-giving. Infant-initiated non-need-based (88%) interactions were more prevalent compared to need-based interactions (12%). Among the non-need-based interactions, 50% aimed at expressing or sharing attention or emotion, 26% aimed at requesting an object or an action, and 12% aimed at seeking information or help. Infant-initiated information-giving events were rare. We further investigated the effects of familial SES and infant sex, finding no effect of either on the number of infant-initiated interactions. These findings suggest that at 18 months, infants actively communicate with their social partners to fulfil their need-based and non-need-based motivations using a wide range of verbal and nonverbal behaviors, regardless of their sex and socio-economic background. This study thoroughly characterizes a wide and detailed range of infant-initiated spontaneous communicative bids in hard-to-access contexts (infants' daily lives at home) and with a traditionally underrepresented non-WEIRD population.


Subject(s)
Gestures , Infant Behavior , Infant , Humans , Infant Behavior/physiology , Intention , Emotions , Attention/physiology
3.
J Pediatr Hematol Oncol ; 45(7): e899-e903, 2023 10 01.
Article in English | MEDLINE | ID: mdl-37278557

ABSTRACT

INTRODUCTION: Propranolol, a nonselective beta-blocker used in the medical treatment of infantile Hemangioma (IH), has been shown to decrease the levels of vascular endothelial growth factor and reduce angiogenesis with its antiproliferative and antiangiogenetic effects. MATERIALS AND METHODS: It has been reported that the storage, transport, and secretion of vascular endothelial growth factor (VEGF) are associated with platelet volume indices (PVI). We aimed to investigate the effect of propranolol on PVI in IH patients. Propranolol treatment was started on 22 IH patients. Platelets, mean platelet volume (MPV), platelet distribution width (PDW), and plateletcrit values in the follow-ups at months 0, 1, and 2 were compared between 22 patients who received treatment and 25 patients who did not. RESULTS: While a statistically significant difference between months 0, 1, and 2 in PDW and MPV values was detected in the treated group, it was not detected in the untreated group. Taking into consideration that VEGF levels were higher at the beginning of the treatment in the pathophysiology of the disease, it was thought that the decrease in VEGF levels by propranolol may have led to a decrease in MPV and PDW levels in the treatment group. CONCLUSION: Consequently, in IH cases, propranolol response follow-up can be evaluated with PVIs, especially MPV and PDW, and it may facilitate clinicians' monitoring of the disease after propranolol administration.


Subject(s)
Hemangioma , Propranolol , Humans , Propranolol/therapeutic use , Vascular Endothelial Growth Factor A/metabolism , Blood Platelets/metabolism , Mean Platelet Volume , Hemangioma/drug therapy
4.
J Pediatr Hematol Oncol ; 45(6): e746-e749, 2023 08 01.
Article in English | MEDLINE | ID: mdl-36898054

ABSTRACT

The purpose of the study was to review the clinical and pathologic characteristics and treatment results of children with precursor B-cell lymphoblastic lymphoma. Of 530 children diagnosed with non-Hodgkin lymphomas between 2000 and 2021, 39 (7.4%) were identified as having precursor B-cell lymphoblastic lymphoma. Clinical characteristics, pathologic, radiologic, laboratory data, treatments, responses, and overall outcomes were recorded from hospital files and analyzed. The median age of 39 patients (males/females, 23/16) was 8.3 years (range 1.3 to 16.1). The most common sites of involvement were the lymph nodes. At a median follow-up of 55.8 months, 14 patients (35%) had a recurrence of disease (11 stage IV, 3 stage III); 4 were in complete remission with salvage therapies, 9 died of progressive disease and one died due to febrile neutropenia. Five-year event-free survival and overall survival rates were 65.4% and 78.3% for all cases, respectively. Survival rates were higher in patients with a complete remission at the end of induction therapies. The survival rates were lower in our study compared with other studies, which could be explained by the high relapse rate and higher incidence of advanced-stage disease due to bone marrow involvement. We demonstrated a prognostic impact of treatment response at the end of the induction phase. Cases with a disease relapse have poor prognosis.


Subject(s)
Lymphoma, B-Cell , Precursor B-Cell Lymphoblastic Leukemia-Lymphoma , Humans , Child , Male , Female , Infant , Child, Preschool , Adolescent , Precursor B-Cell Lymphoblastic Leukemia-Lymphoma/drug therapy , Neoplasm Recurrence, Local/drug therapy , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Lymphoma, B-Cell/pathology , Prognosis , Treatment Outcome , Disease-Free Survival
5.
North Clin Istanb ; 9(3): 241-247, 2022.
Article in English | MEDLINE | ID: mdl-36199858

ABSTRACT

OBJECTIVE: Neuropathic pain (NP) is caused by damage or disease affecting the somatosensory nervous system. The aim of this study was to evaluate the clinical characteristics, pathophysiologies, and treatments applied in pediatric cancer patients with NP. METHODS: Patients with cancer having NP between 5 and 18 years of age who were followed up in the pediatric oncology clinic of Okmeydani Training and Research Hospital between January 2015 and April 2019 were included in this study. NP was described as tingling, burning, and stinging. Patients with acute lymphoblastic leukemia and brain tumors were excluded from the study. A number of pediatric cancer patients were also recorded. Patients' age, gender, cancer diagnosis, NP characteristics and causes, treatments, and response to those treatments were investigated retrospectively and groups of NP according to their pathophysiological mechanism were established. RESULTS: NP was found in 26 (16%) of 160 patients followed up for childhood cancers. The average age was 11.8±4 years. Ten of the patients (38.4%) were female, and 16 (61.5%) were male. Osteosarcoma was the most common diagnosis in 10 (38%) patients. The most common cause of NP was compression of a nerve/root/spinal cord in 9 (35%) patients and the second most common was related with limb-sparing surgery. NP was found to be associated with chemotherapy (CT) in 5 (19%) patients, mostly with vincristine. Gabapentin was administered in a total of 22 (85%) patients for treatment. Opioid administration was more common as the disease stage progressed (p<0.05). A good or partial response to treatment was achieved in 19 (73%) patients. CONCLUSION: NP can occur in childhood cancers and is related to the cancer itself, CT, surgical treatment, and disseminated disease. Although there is no standard protocol, gabapentin and, for advanced-stage patients, opioids are the most commonly used treatment options.

6.
Front Psychol ; 13: 1015649, 2022.
Article in English | MEDLINE | ID: mdl-36817372

ABSTRACT

Infants are drawn to events that violate their expectations about the world: they look longer at physically impossible events, such as when a car passes through a wall. Here, we examined whether individual differences in infants' visual preferences for physically impossible events reflect an early form of curiosity, and asked whether caregivers' behaviors, parenting styles, and everyday routines relate to these differences. In Study 1, we presented infants (N = 47, Mage = 16.83 months, range = 10.29-24.59 months) with events that violated physical principles and closely matched possible events. We measured infants' everyday curiosity and related experiences (i.e., caregiver curiosity-promoting activities) through a newly developed curiosity scale, The Early Multidimensional Curiosity Scale (EMCS). Infants' looking preferences for physically impossible events were positively associated with their score on the EMCS, but not their temperament, vocabulary, or caregiver trait curiosity. In Study 2A, we set out to better understand the relation between the EMCS and infants' looking preferences for physically impossible events by assessing the underlying structure of the EMCS with a larger sample of children (N = 211, Mage = 47.63 months, range = 10.29-78.97 months). An exploratory factor analysis revealed that children's curiosity was comprised four factors: Social Curiosity, Broad Exploration, Persistence, and Information-Seeking. Relatedly, caregiver curiosity-promoting activities were composed of five factors: Flexible Problem-Solving, Cognitive Stimulation, Diverse Daily Activities, Child-Directed Play, and Awe-Inducing Activities. In Study 2B (N = 42 infants from Study 1), we examined which aspects of infant curiosity and caregiver behavior predicted infants' looking preferences using the factor structures of the EMCS. Findings revealed that infants' looking preferences were uniquely related to infants' Broad Exploration and caregivers' Awe-Inducing Activities (e.g., nature walks with infants, museum outings). These exploratory findings indicate that infants' visual preferences for physically impossible events may reflect an early form of curiosity, which is related to the curiosity-stimulating environments provided by caregivers. Moreover, this work offers a new comprehensive tool, the Early Multidimensional Curiosity Scale, that can be used to measure both curiosity and factors related to its development, starting in infancy and extending into childhood.

7.
Pediatr Hematol Oncol ; 39(2): 121-131, 2022 Mar.
Article in English | MEDLINE | ID: mdl-34281453

ABSTRACT

The longer diagnostic intervals in low- and middle-income countries have been proposed among the possible causes of poorer outcomes in children with cancer. In this single-center study from Turkey, the diagnostic intervals and survival status of 138 children with solid tumors and lymphoma (excluding leukemia) were prospectively evaluated. The median total interval (from the beginning of the first cancer-related symptom to the first day of the cancer-specific therapy), the median patient interval (the time interval from the notification of the first cancer-related symptom to the first admission to a healthcare facility), and the median physician interval (the time interval between the first healthcare admission to the first pediatric oncology visit) were 65, 26, and 24 days, respectively. The estimated 5-year overall survival and event-free survival rates were 80.7% and 69.1%, respectively. The longer time intervals were correlated with age, paternal education, localization, and tumor type. Interestingly, none of the time parameters were found to be associated with survival on regression analysis. In conclusion, the diagnostic delay in children with cancer is multifactorial, and the patient- and disease-related factors are as important as the time intervals on survival.Supplemental data for this article is available online at https://doi.org/10.1080/08880018.2021.1951903.


Subject(s)
Lymphoma , Neoplasms , Child , Delayed Diagnosis , Humans , Lymphoma/diagnosis , Lymphoma/therapy , Neoplasms/diagnosis , Neoplasms/therapy , Time Factors , Turkey/epidemiology
8.
Turk J Pediatr ; 63(3): 363-371, 2021.
Article in English | MEDLINE | ID: mdl-34254481

ABSTRACT

BACKGROUND: This study aims to evaluate the etiology of cervical lymphadenopathies in children and to define the significance of demographic, clinical, and laboratory features in the prediction of malignancy. METHODS: Medical records of 527 patients were reviewed retrospectively between 2015 and 2019. The patients were examined in terms of demographics, clinical, radiologic, and serologic findings. A lymph node biopsy was performed in selected patients. The risk factors for malignancy were evaluated. RESULTS: Out of 527 children, 26 had neck masses mimicking lymphadenopathy; 501 had lymphadenopathy. The most common location was the anterior cervical region and the median age was 5.7 years. Thirty-nine patients had malignancy (lymphoma in 34, nasopharyngeal carcinoma in 3, leukemia in 1 and neuroblastoma in 1). The risk of malignancy was associated with older age, duration of > 4 weeks, lymph node size > 3 cm, supraclavicular location, presence of systemic symptoms, and hepatosplenomegaly (p < 0.001, p < 0.001, p < 0.001, p < 0.001, p < 0.001, p < 0.001). On laboratory evaluation, anemia, leukocytosis, and increased erythrocyte sedimentation rate were found to be associated with malignancy (p < 0.001, p=0.003, p < 0.001). CONCLUSIONS: Cervical lymphadenopathies in children are generally benign but patients with persisting cervical lymphadenopathy, adolescent age, accompanying systemic symptoms and abnormal laboratory findings should be considered for an early biopsy.


Subject(s)
Lymphadenopathy , Lymphatic Diseases , Adolescent , Aged , Biopsy , Child , Child, Preschool , Humans , Lymph Nodes , Lymphadenopathy/diagnosis , Lymphadenopathy/epidemiology , Lymphadenopathy/etiology , Lymphatic Diseases/diagnosis , Lymphatic Diseases/epidemiology , Lymphatic Diseases/etiology , Retrospective Studies
9.
Naunyn Schmiedebergs Arch Pharmacol ; 394(8): 1769-1777, 2021 08.
Article in English | MEDLINE | ID: mdl-34151393

ABSTRACT

Relapsed or refractory central nervous system (CNS) tumors still have poor prognosis, and, therefore, new treatment options are required. We retrospectively researched treatment results of patients with CNS tumors treated with nimotuzumab from 2010 to 2015. The study included nine patients with the diffuse intrinsic pontine glioma; eight with medulloblastoma; three each with anaplastic ependymoma, glioblastoma multiforme, and central nervous system primitive neuroectodermal tumor (CNS PNET); two patients with gliomatosis cerebri; and one patient each with other tumor types, including atypical teratoid rhabdoid tumor, thalamic astrocytoma, low-grade glial tumor, high-grade glial tumor, and cribriform neuroepithelial tumor. An objective response was observed in 10 of 33 patients with four patients showing a complete response, three a partial response, and three patients had stable disease. The 2-year overall survival (OS) and progression-free survival (PFS) rates were 35 ±9% and 19 ±8%, respectively. Due to the objective response in medulloblastoma, CNS PNET, and anaplastic ependymoma (MED group), survival rates of this group were analyzed. The 2-year OS and PFS for the MED group were 71 ±12% and 30 ±13%, respectively. The treatment was well tolerated. The treatment responses for medulloblastoma, CNS PNET, and anaplastic ependymoma have been promising. Likewise, some patients with relapsed or progressive CNS tumors may benefit through nimotuzumab-containing regimen.


Subject(s)
Antibodies, Monoclonal, Humanized/administration & dosage , Antineoplastic Agents, Immunological/administration & dosage , Central Nervous System Neoplasms/drug therapy , Adolescent , Antibodies, Monoclonal, Humanized/adverse effects , Antibodies, Monoclonal, Humanized/pharmacology , Antineoplastic Agents, Immunological/pharmacology , Central Nervous System Neoplasms/pathology , Child , Child, Preschool , Female , Humans , Infant , Male , Progression-Free Survival , Retrospective Studies , Survival Rate , Treatment Outcome
10.
Eurasian J Med ; 53(1): 5-8, 2021 Feb.
Article in English | MEDLINE | ID: mdl-33716522

ABSTRACT

OBJECTIVE: After chemotherapy, cancer survivors suffer from acquired immunological defects and become vulnerable to vaccine-preventable diseases. There are no universally approved revaccination guidelines for non-transplanted oncology patients. This study aimed to share our experience of revaccination in childhood cancer survivors to plan future vaccination schedules. MATERIALS AND METHODS: This retrospective study was conducted in a Pediatric Oncology Department of a university-affiliated hospital. Patients who were diagnosed with malignancy other than leukemia constituted the study population. Patients were directed for revaccination 6 months after the cessation of treatment. Revaccination was performed according to patients' vaccination status before chemotherapy and seronegativity. RESULTS: Of the 64 patients in the study, 44 (68.75%) were boys. The mean age at the time of diagnosis and at start of vaccination was 8.8±5.3 years and 10.6±5.1 years, respectively. Hodgkin's lymphoma was the most common diagnosis. The vaccination schedule of 7 patients was interrupted because of chemotherapy; after completing the missing vaccine doses, the serology of 2 patients was negative for at least 2 antigens. The vaccination schedule of 57 patients was completed before beginning chemotherapy and 52 of them were seronegative for at least 1 antigen. No adverse reactions or life-threatening infections were observed because of vaccinations. CONCLUSION: There are different approaches when vaccinating the oncology patients after chemotherapy. Watching out for the four touchstones mentioned in our study will protect the patient and do no harm. More studies are needed to constitute universal and standardized revaccination guidelines for these patients.

11.
Dev Psychol ; 57(2): 284-301, 2021 Feb.
Article in English | MEDLINE | ID: mdl-33346676

ABSTRACT

Maternal control is a major dimension of parenting and has different meanings, practices, and potential consequences across cultures. The present study aimed to identify and compare mothers' conceptualizations of parenting control across four cultures to reveal a more nuanced understanding regarding the meaning and practices of control: European American, Chinese immigrant, Korean immigrant, and Turkish. Using a semistructured open-ended interview, 100 European American, 102 U.S. Chinese immigrant, 103 U.S. Korean immigrant, and 109 Turkish mothers of preschool-aged children reported the ratings of importance, specific reasons, and strategies for exerting control over their children in daily life. Results revealed both shared and unique conceptualizations of maternal control across four cultures. Specifically, all mothers reported that it is important to express maternal control over their children in order to set behavioral norms/standards, maintain child safety, support social relations and respect for others, provide guidance, and guide moral development. Moreover, mothers discussed utilizing nonphysical punishment, setting and maintaining rules, reasoning/negotiating, consistency, physical punishment and verbal control, showing parents' serious/stern attitude, correction, and psychological control forms of control. However, the levels at which mothers emphasize the different reasons and strategies varied across cultures, reflecting culturally emphasized values. The findings of the present study further enrich our understanding of the complexities of maternal control across cultures. (PsycInfo Database Record (c) 2021 APA, all rights reserved).


Subject(s)
Emigrants and Immigrants , Parenting , Child , Child, Preschool , Female , Humans , Mothers , Pregnancy , Punishment , White People
12.
Turk J Pediatr ; 62(6): 979-985, 2020.
Article in English | MEDLINE | ID: mdl-33372436

ABSTRACT

BACKGROUND: Infantile hemangiomas (IH) represent the most common type of benign tumors of infancy. Vascular endothelial growth factor (VEGF) and basic fibroblastic growth factor (bFGF) have a central role in the pathogenesis of infantile hemangiomas. METHODS: In this prospective study, we aimed to investigate the relationship between serum VEGF and bFGF levels and clinical characteristics and the serological changes in VEGF and bFGF levels associated with propranolol treatment in infants diagnosed with IH. Blood samples were taken from 34 patients with IH and 10 controls. Serum VEGF and bFGF levels were studied by ELISA. RESULTS: At initial diagnosis, median serum bFGF levels were 11.1 ng/ml (4.8-16.6) in patients with IH (n=34) and 2.6 ng/ml (1.7-4.7) in controls (p < 0.001), and, median serum VEGF levels for same groups were 58.5 ng/ml (25.3-190.2) and 11.4 ng/ml (8.2-19.8) (p < 0.001), respectively. Serum VEGF and bFGF levels were not correlated. In 18 infants who were treated with propranolol with serial measurements, median serum bFGF levels were 10.7 ng/ml, 9.8 ng/ml and 10.5 ng/ml (p= 0.8), and median serum VEGF levels were 68.6 ng/ml, 63.5 ng/ml and 45.1 ng/ml (p < 0.001) at initial diagnosis, at first and third months, respectively. Median regression rates of the hemangiomas at the first and third months were -%47.3 and -%58.3 (p < 0.001), respectively. CONCLUSIONS: Serum bFGF levels didn`t change in time. Serum VEGF levels seemed to follow the natural course of IH and might be a marker for follow-up. The contribution of propranolol treatment should also be considered.


Subject(s)
Hemangioma , Vascular Endothelial Growth Factor A , Fibroblast Growth Factor 2 , Hemangioma/drug therapy , Humans , Infant , Propranolol , Prospective Studies , Vascular Endothelial Growth Factors
13.
Turk J Pediatr ; 62(5): 858-862, 2020.
Article in English | MEDLINE | ID: mdl-33108091

ABSTRACT

BACKGROUND: Kaposi`s sarcoma (KS) is a complication of immunosuppressive therapy for transplant recipients. Unlike adult recipients, KS in pediatric organ transplantation is quite rare. Treatment is usually withdrawal of immunosuppression; non-responders often receive chemotherapy. CASE: We have reported a child with post-liver transplant visceral KS which has progressed despite withdrawal of immunosuppressive therapy, who has been treated with Paclitaxel for three weeks. KS has regressed completely after four cycles of Paclitaxel. CONCLUSION: Paclitaxel should be considered as an effective first line treatment option for patients with posttransplant KS.


Subject(s)
Liver Transplantation , Sarcoma, Kaposi , Adult , Child , Humans , Immunosuppression Therapy , Paclitaxel , Sarcoma, Kaposi/drug therapy , Transplant Recipients
14.
Asian J Surg ; 43(1): 207-212, 2020 Jan.
Article in English | MEDLINE | ID: mdl-30962018

ABSTRACT

BACKGROUND/OBJECTIVE: This study aims to evaluate the current surgical approach to adrenal masses in the pediatric age group. METHODS: We retrospectively analyzed cases that underwent surgery for adrenal masses between 2007 and 2017. Patients were assessed regarding age, sex, primary diagnosis, image defined risk factors (IDRF), surgical treatment method, complications, duration of hospital stay, and follow-up. RESULTS: We examined 50 patients who underwent surgery for adrenal mass (mean age: 4.8 years; range: 5 days-14 years). For IDRF assessment, Ultrasonography was used in 42, Computed Tomography in 36, and Magnetic Resonance Imaging in 36 patients. Lesions were present on the right in 25, left in 21, and bilateral in 4 patients. Histopathological findings were neuroblastoma (n = 29), ganglioneuroma (n = 6), adrenal cortex tumor (n = 5), ganglioneuroblastoma (n = 4), pheochromocytoma (n = 3), cyst (n = 1), and adrenal hematoma (n = 2). Laparotomy was performed on 37 patients, and laparoscopy on 13 patients. None of the cases had any operative complications. CONCLUSION: The crucial factors determining the surgical approach to adrenal masses in pediatric cases are the histopathology of the mass, volume, and IDRF. Minimally invasive procedures could be reliably performed in appropriate cases.


Subject(s)
Adrenal Cortex Neoplasms/surgery , Adrenal Gland Diseases/surgery , Adrenal Gland Neoplasms/surgery , Ganglioneuroblastoma/surgery , Ganglioneuroma/surgery , Hematoma/surgery , Neuroblastoma/surgery , Pheochromocytoma/surgery , Adolescent , Adrenal Cortex Neoplasms/diagnostic imaging , Adrenal Cortex Neoplasms/pathology , Adrenal Gland Diseases/diagnostic imaging , Adrenal Gland Diseases/pathology , Adrenal Gland Neoplasms/diagnostic imaging , Adrenal Gland Neoplasms/pathology , Animals , Child , Child, Preschool , Female , Ganglioneuroblastoma/diagnostic imaging , Ganglioneuroblastoma/pathology , Ganglioneuroma/diagnostic imaging , Ganglioneuroma/pathology , Hematoma/diagnostic imaging , Hematoma/pathology , Hemiptera , Humans , Infant , Male , Neuroblastoma/diagnostic imaging , Neuroblastoma/pathology , Pheochromocytoma/diagnostic imaging , Pheochromocytoma/pathology , Retrospective Studies , Risk Factors
15.
Indian Pediatr ; 54(1): 53-54, 2017 Jan 15.
Article in English | MEDLINE | ID: mdl-28141567

ABSTRACT

BACKGROUND: Blue rubber bleb nevus syndrome is a rare disease involving venous malformations. CASE CHARACTERISTICS: We present a 6-year-old female with the syndrome, and consumptive coagulopathy. INTERVENTION/OUTCOME: After management with sirolimus, symptoms resolved. MESSAGE: Sirolimus may be a valuable option for reducing bleeding complications and cosmetic sequelae for the patients with this syndrome.


Subject(s)
Antibiotics, Antineoplastic/therapeutic use , Gastrointestinal Neoplasms/drug therapy , Gastrointestinal Neoplasms/pathology , Nevus, Blue/drug therapy , Nevus, Blue/pathology , Sirolimus/therapeutic use , Skin Neoplasms/drug therapy , Skin Neoplasms/pathology , Child , Female , Hemangioma , Humans , Skin/pathology
16.
Pediatr Int ; 59(5): 545-550, 2017 May.
Article in English | MEDLINE | ID: mdl-28083971

ABSTRACT

BACKGROUND: Lipoblastoma is a rare benign mesenchymal tumor of infancy and early childhood. Symptoms vary depending on localization, and signs of compression of adjacent organs may be seen. METHODS: A total of 12 children with pathologically proven lipoblastoma from 2000 to 2014 were reviewed retrospectively for their clinical features, treatment and follow up. RESULTS: There were seven boys and five girls between 7.5 months and 7.5 years of age. The most common symptom was painless rapid-growing mass. The tumors were located in the neck, the abdomen, the groin, pelvis, axilla, glutea, labium majus, thigh and trunk. The largest tumor was in a retroperitoneal location and was 13 × 10 cm in size. Complete resection was performed in 10 patients. All of these patients were followed without any evidence of recurrence. Subtotal excision was performed in two patients. At a median follow up of 38 months (range, 1.8 months-10 years), all patients were disease free. CONCLUSIONS: Lipoblastoma should be considered in the differential diagnosis of rapidly growing mass in patients <3 years of age. Total excision is the treatment of choice with preservation of vital organs. The prognosis is excellent despite large tumor size and local invasion. Recurrence rate is high in incompletely resected tumors. Regular follow up is important for early detection of recurrence.


Subject(s)
Lipoblastoma/diagnosis , Lipoblastoma/surgery , Child , Child, Preschool , Diagnosis, Differential , Female , Follow-Up Studies , Humans , Infant , Lipoblastoma/pathology , Male , Retrospective Studies , Treatment Outcome
17.
J Pediatr Hematol Oncol ; 38(4): e147-50, 2016 05.
Article in English | MEDLINE | ID: mdl-26989912

ABSTRACT

Wilms tumor is the most common renal malignancy of childhood. Because of the improvement in prognosis and the increase in survival rates, long-term consequences of the treatment for Wilms tumor are of greater concern. We investigated late renal effects of the treatment on 50 survivors of nonsyndromic unilateral Wilms tumor. After the second year since the cessation of treatment, the glomerular filtration rate (GFR), urinary protein excretion, urinary ß2 microglobulin levels, and blood pressure as well as the general health status were assessed. Results were analyzed for correlation with clinical variables, chemotherapy, and radiotherapy as possible risk factors. At a median follow-up time of 8.8 years (mean=10.9; range, 2.3 to 35.4 y), none of the patients developed end-stage renal disease. Compensatory hypertrophy was observed in 68% of the cases. The median maximum bipolar length was significantly higher in patients diagnosed after the age of 36 months. Eleven (22%) and 2 (4%) of the 50 patients were hypertensive at the time of the diagnosis and the study, respectively. Similarly, median GFR values were significantly lower at the time of diagnosis, although at the time of the study, all patients had normal GFR values. With longer follow-up intervals, especially after 10 years, a significant decreasing trend in the GFR was observed (P=0.002).


Subject(s)
Kidney/pathology , Wilms Tumor/complications , Adolescent , Adult , Blood Pressure , Child , Child, Preschool , Follow-Up Studies , Glomerular Filtration Rate , Health Status , Humans , Hypertrophy , Infant , Infant, Newborn , Kidney/drug effects , Kidney/physiopathology , Kidney Neoplasms , Middle Aged , Proteinuria , Time Factors , Young Adult
19.
Turk J Pediatr ; 58(6): 683-686, 2016.
Article in English | MEDLINE | ID: mdl-29090887

ABSTRACT

Fibrous hamartoma of infancy (FHI) is a rare, benign lesion which is commonly seen under two years of age. Clinical and radiological features of FHI can mimic malignant soft tissue sarcomas, it is important to make differential diagnosis. Ultrasound (US) examination reveals heterogeneous echogenicity that can be also suggestive for other soft tissue tumors but newly defined "serpentine pattern" of intervening hypoechoic portions in the hyperechoic mass with poorly defined margins and with poor vascularity is special for FHI. Here we report a 15-month-old-boy with FHI with serpentine pattern on US. He initially presented with a painless mass in his left axilla existing for approximately seven months. The mass was successfully excised and he has been followed for three years without any evidence of recurrence. Fibrous hamartoma of infancy should always be considered in differential diagnosis in children under two years of age with a firm and solitary mass in the axilla especially when US reveals serpentine pattern with poorly defined margins and with poor vascularity. If these clinical and ultrasonographic findings are seen in a child under two years old, surgery can be performed without any additional imaging modalities. Awareness and careful assessment are important in order not to misdiagnose this benign mass for which surgical excision is curative.

20.
Ophthalmologica ; 234(4): 227-32, 2015.
Article in English | MEDLINE | ID: mdl-26368674

ABSTRACT

BACKGROUND: Studies conducted in recent years have reported promising results regarding the treatment of retinoblastoma with the intra-arterial use of melphalan. In the present study, we intended to report the results of intra-arterial chemotherapy with melphalan (IACT) in the treatment of newly diagnosed or relapsed-refractory retinoblastoma patients at the Department of Pediatric Oncology of Hacettepe University, Ankara, Turkey. MATERIALS AND METHODS: This was a retrospective study of patients with intraocular retinoblastoma who were treated with IACT from December 2011 to May 2014. A total of 56 eyes of 46 consecutive patients (30 males and 16 females) were included in the study. Forty-four eyes received systemic chemotherapy upon diagnosis (systemic chemotherapy group, SCG), and 12 eyes were those of newly diagnosed patients (primary intra-arterial melphalan group, PIAG). The choice of the IACT dose was based on age. Tumor control and globe salvage with IACT were analyzed. Complete blood counts were examined 7 days after the IACT for systemic toxicity. Ocular toxicities such as proptosis, eyelid edema, ocular motility, and retinal and optic atrophy were assessed by an ocular oncologist with regular ophthalmologic examinations. RESULTS: Enucleation was avoided overall in 66% (37/56) of the eyes, including 75% (9/12) in the PIAG and 64% (28/44) in the SCG patients. The 1-year enucleation-free survival rate was 56.7% at a median follow-up time of 11.9 months (range 0.27-27.6). IACT was administered in a total of 124 cycles (ranging from 1 to 7 cycles, mean 2.3). The responses were as follows: regression of the retinal tumor in 27 eyes and improvements in vitreous seeding in 5 of 15 eyes. The further treatment requirements after IACT were as follows: enucleation in 19 eyes (10 with vitreous seeding), radiotherapy in 3 eyes, systemic chemotherapy in 1 eye, and local therapy in 1 eye. No severe systemic side effects occurred. Transient swelling of the eyelids (22 patients), conjunctival chemosis (12 patients), upper eyelid ptosis (5 patients), redness over the frontal area (3 patients), limitation of ocular motility (3 patients) and mild proptosis (1 patient) were detected. Retinal pigment epithelial alterations (30 patients) and optic atrophy (3 patients) were seen in the late follow-up. CONCLUSIONS: Globe salvage and avoidance of radiotherapy may be achieved by IACT with limited toxicity. This treatment is efficient, repeatable and safe.


Subject(s)
Antineoplastic Agents, Alkylating/administration & dosage , Melphalan/administration & dosage , Neoplasm Recurrence, Local/drug therapy , Retinal Neoplasms/drug therapy , Retinoblastoma/drug therapy , Antineoplastic Agents, Alkylating/therapeutic use , Child , Child, Preschool , Disease-Free Survival , Eye Enucleation , Female , Humans , Infant , Infusions, Intra-Arterial , Male , Melphalan/therapeutic use , Retinal Neoplasms/mortality , Retinal Neoplasms/pathology , Retinoblastoma/mortality , Retinoblastoma/pathology , Retrospective Studies , Survival Rate
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