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  1. Article ; Online: SARS-CoV-2 infection in pediatric patient with hemoglobin SC disease.

    Dagalakis, Urania / Hammershaimb, Elizabeth / McArthur, Monica A / Macatangay, Regina A

    Pediatric blood & cancer

    2020  Volume 67, Issue 11, Page(s) e28430

    MeSH term(s) Acute Chest Syndrome/epidemiology ; Anti-Bacterial Agents/therapeutic use ; COVID-19/complications ; COVID-19/diagnosis ; COVID-19/drug therapy ; Ceftriaxone/therapeutic use ; Hemoglobin SC Disease/complications ; Humans ; Infant ; Male ; SARS-CoV-2 ; Tomography, X-Ray Computed ; Treatment Outcome
    Chemical Substances Anti-Bacterial Agents ; Ceftriaxone (75J73V1629)
    Keywords covid19
    Language English
    Publishing date 2020-08-09
    Publishing country United States
    Document type Case Reports ; Letter
    ZDB-ID 2131448-2
    ISSN 1545-5017 ; 1545-5009
    ISSN (online) 1545-5017
    ISSN 1545-5009
    DOI 10.1002/pbc.28430
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  3. Article ; Online: SARS-CoV-2 infection in pediatric patient with hemoglobin SC disease

    Dagalakis, Urania / Hammershaimb, Elizabeth / McArthur, Monica A. / Macatangay, Regina A.

    2020  

    Abstract: https://doi.org/10.1002/pbc ... ...

    Abstract https://doi.org/10.1002/pbc.28430
    Keywords COVID-19 ; Hemoglobin SC Disease ; Infant ; Case Reports ; covid19
    Language English
    Publishing date 2020-08-09
    Publisher Wiley-Blackwell
    Publishing country us
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  4. Article ; Online: SARS‐CoV‐2 infection in pediatric patient with hemoglobin SC disease

    Dagalakis, Urania / Hammershaimb, Elizabeth / McArthur, Monica A. / Macatangay, Regina A.

    Pediatric Blood & Cancer

    2020  Volume 67, Issue 11

    Keywords Pediatrics, Perinatology, and Child Health ; Oncology ; Hematology ; covid19
    Language English
    Publisher Wiley
    Publishing country us
    Document type Article ; Online
    ZDB-ID 2131448-2
    ISSN 1545-5017 ; 1545-5009
    ISSN (online) 1545-5017
    ISSN 1545-5009
    DOI 10.1002/pbc.28430
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  5. Article: Positive fertility outcomes in a female with classic congenital adrenal hyperplasia following bilateral adrenalectomy.

    Dagalakis, Urania / Mallappa, Ashwini / Elman, Meredith / Quezado, Martha / Merke, Deborah P

    International journal of pediatric endocrinology

    2016  Volume 2016, Page(s) 10

    Abstract: Background: Classic congenital adrenal hyperplasia (CAH) requires lifetime steroid replacement and supraphysiologic glucocorticoid dose is often required for adequate adrenal androgen suppression. Patients often suffer from long-term co-morbidities and ... ...

    Abstract Background: Classic congenital adrenal hyperplasia (CAH) requires lifetime steroid replacement and supraphysiologic glucocorticoid dose is often required for adequate adrenal androgen suppression. Patients often suffer from long-term co-morbidities and female infertility is common.
    Case presentation: We report the use of laparoscopic bilateral adrenalectomy as a treatment for a 21 year old female with classic simple virilizing CAH and infertility. She presented as an adolescent with increasing weight gain, amenorrhea and elevated adrenal androgens despite the use of dexamethasone (250 mcg given twice daily), and fludrocortisone (150 mcg daily). An anti-androgen (flutamide 250 mg given twice daily) and a combined oral contraceptive pill were added to her regimen and prevented progressive virilization, but she eventually desired fertility. A bilateral laparoscopic adrenalectomy was performed at age 21. The right adrenal gland weighed 41.8 grams and the left gland 45.5 grams. There were no complications during the surgery. Since the surgery, she has had a total of three pregnancies, resulting in 3 healthy full-term infants. Follow-up 7 years later at age 27 revealed overall excellent health with a BMI of 25.1 kg/m(2), no evidence of adrenal rest tissue based on hormonal testing, above average quality-of-life based on 36-item short-form health survey and she has not experienced an adrenal crisis.
    Conclusions: This case highlights the use of bilateral adrenalectomy as a treatment option for female infertility in a patient with classic CAH and difficult-to-control hyperandrogenism secondary to adrenal nodular hyperplasia. Outstanding quality-of-life, disease control and fertility were achieved.
    Language English
    Publishing date 2016-05-20
    Publishing country England
    Document type Case Reports
    ZDB-ID 2528691-2
    ISSN 1687-9856 ; 1687-9848
    ISSN (online) 1687-9856
    ISSN 1687-9848
    DOI 10.1186/s13633-016-0028-4
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  6. Article ; Online: Puberty and plexiform neurofibroma tumor growth in patients with neurofibromatosis type I.

    Dagalakis, Urania / Lodish, Maya / Dombi, Eva / Sinaii, Ninet / Sabo, Jessica / Baldwin, Andrea / Steinberg, Seth M / Stratakis, Constantine A / Widemann, Brigitte C

    The Journal of pediatrics

    2013  Volume 164, Issue 3, Page(s) 620–624

    Abstract: Objective: To assess the relationship between pubertal progression and change in plexiform neurofibroma (PN) burden over time in pediatric and young adult patients with neurofibromatosis type 1 and PNs.: Study design: Analyses accounted for sex, age, ...

    Abstract Objective: To assess the relationship between pubertal progression and change in plexiform neurofibroma (PN) burden over time in pediatric and young adult patients with neurofibromatosis type 1 and PNs.
    Study design: Analyses accounted for sex, age, race, and chemotherapy. Forty-one patients with neurofibromatosis type 1 (15 female and 26 male patients) were studied at the National Institutes of Health. Tanner stage, testosterone, progesterone, estradiol, insulin-like growth factor -1, luteinizing hormone, and follicle-stimulating hormone were assessed. Tumor volume was measured using magnetic resonance imaging and lesion detection software developed locally. Patients were divided into 2 groups based on whether they were actively progressing through puberty (n = 16) or were peripubertal (n = 25) and were followed for an average of 20 months. Tumor growth rates in the puberty and peripubertal group were analyzed for a subset of patients.
    Results: There was no statistically significant difference in tumor burden change over time (cm(2)/kg per month) between the pubertal and peripubertal groups (-0.16 ± 0.34 vs 0.03 ± 1.8, P = .31) and in the PN growth rates before and during puberty (P = .90). Change in tumor volume/patient weight/time did not correlate with testosterone change/time in males or estradiol change/time in females.
    Conclusion: These findings support that hormonal changes of puberty do not accelerate PN growth. Additional long-term follow-up of patients is necessary to further characterize the interaction between puberty and tumor growth.
    MeSH term(s) Adolescent ; Child ; Estrogens/blood ; Female ; Follicle Stimulating Hormone/blood ; Follow-Up Studies ; Humans ; Insulin-Like Growth Factor I/analysis ; Luteinizing Hormone/blood ; Magnetic Resonance Imaging ; Male ; Neurofibroma, Plexiform/pathology ; Neurofibromatosis 1/pathology ; Progesterone/blood ; Prospective Studies ; Puberty ; Testosterone/blood
    Chemical Substances Estrogens ; Testosterone (3XMK78S47O) ; Progesterone (4G7DS2Q64Y) ; Insulin-Like Growth Factor I (67763-96-6) ; Luteinizing Hormone (9002-67-9) ; Follicle Stimulating Hormone (9002-68-0)
    Language English
    Publishing date 2013-12-08
    Publishing country United States
    Document type Comparative Study ; Journal Article ; Observational Study ; Research Support, N.I.H., Intramural
    ZDB-ID 3102-1
    ISSN 1097-6833 ; 0022-3476
    ISSN (online) 1097-6833
    ISSN 0022-3476
    DOI 10.1016/j.jpeds.2013.10.081
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: Bone mineral density in children and young adults with neurofibromatosis type 1.

    Lodish, Maya B / Dagalakis, Urania / Sinaii, Ninet / Bornstein, Ethan / Kim, Aerang / Lokie, Kelsey B / Baldwin, Andrea M / Reynolds, James C / Dombi, Eva / Stratakis, Constantine A / Widemann, Brigitte C

    Endocrine-related cancer

    2012  Volume 19, Issue 6, Page(s) 817–825

    Abstract: Concern for impaired bone health in children with neurofibromatosis type 1 (NF-1) has led to increased interest in bone densitometry in this population. Our study assessed bone mineral apparent density (BMAD) and whole-body bone mineral content (BMC)/ ... ...

    Abstract Concern for impaired bone health in children with neurofibromatosis type 1 (NF-1) has led to increased interest in bone densitometry in this population. Our study assessed bone mineral apparent density (BMAD) and whole-body bone mineral content (BMC)/height in pediatric patients with NF-1 with a high plexiform neurofibroma burden. Sixty-nine patients with NF-1 (age range 5.2-24.8; mean 13.7 ± 4.8 years) were studied. Hologic dual-energy X-ray absorptiometry scans (Hologic, Inc., Bedford, MA, USA) were performed on all patients. BMD was normalized to derive a reference volume by correcting for height through the use of the BMAD, as well as the BMC. BMAD of the lumbar spine (LS 2-4), femoral neck (FN), and total body BMC/height were measured and Z-scores were calculated. Impaired bone mineral density was defined as a Z-score ≤-2. Forty-seven percent of patients exhibited impaired bone mineral density at any bone site, with 36% at the LS, 18% at the FN, and 20% total BMC/height. BMAD Z-scores of the LS (-1.60 ± 1.26) were more impaired compared with both the FN (-0.54 ± 1.58; P=0.0003) and the whole-body BMC/height Z-scores (-1.16 ± 0.90; P=0.036). Plexiform neurofibroma burden was negatively correlated with LS BMAD (r(s)=-0.36, P=0.01). In pediatric and young adult patients with NF-1, LS BMAD was more severely affected than the FN BMAD or whole-body BMC/height.
    MeSH term(s) Adolescent ; Adult ; Bone Density ; Child ; Child, Preschool ; Female ; Femur Neck/physiopathology ; Humans ; Lumbar Vertebrae/physiopathology ; Male ; Neurofibroma, Plexiform/physiopathology ; Neurofibromatosis 1/physiopathology ; Young Adult
    Language English
    Publishing date 2012-11-19
    Publishing country England
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, N.I.H., Intramural
    ZDB-ID 1218450-0
    ISSN 1479-6821 ; 1351-0088
    ISSN (online) 1479-6821
    ISSN 1351-0088
    DOI 10.1530/ERC-12-0293
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.A 4192: Show issues Location:
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  8. Article ; Online: (111)In-octreotide scintigraphy for identification of metastatic medullary thyroid carcinoma in children and adolescents.

    Lodish, Maya / Dagalakis, Urania / Chen, Clara C / Sinaii, Ninet / Whitcomb, Patricia / Aikin, Alberta / Dombi, Eva / Marcus, Leigh / Widemann, Brigitte / Fox, Elizabeth / Chuk, Meredith / Balis, Frank / Wells, Samuel / Stratakis, Constantine A

    The Journal of clinical endocrinology and metabolism

    2011  Volume 97, Issue 2, Page(s) E207–12

    Abstract: Context: Most medullary thyroid cancers (MTC) express somatostatin receptors; therefore, (111)In-octreotide somatostatin receptor scintigraphy (SRS) may be useful in detecting sites of metastases in children with MTC.: Objective: The aim of the study ...

    Abstract Context: Most medullary thyroid cancers (MTC) express somatostatin receptors; therefore, (111)In-octreotide somatostatin receptor scintigraphy (SRS) may be useful in detecting sites of metastases in children with MTC.
    Objective: The aim of the study was to evaluate tumor metastases in children and adolescents with MTC using SRS in comparison to conventional imaging.
    Design and setting: A case series was conducted as part of baseline evaluation for cancer treatment protocol at the National Institutes of Health Clinical Center.
    Patients: Eleven patients with a median age of 15 (range, 9-17) yr participated in the study, 10 with histologically proven, metastatic MTC due to the M918T mutation of the RET protooncogene, and one with a known RET polymorphism.
    Intervention: After receiving 0.086 mCi/kg (111)Indium-pentreotide, patients were examined with a single photon emission computed tomography scan 4 and 24 h after injection. Baseline conventional imaging, including computed tomography (neck, chest, abdomen, ± pelvis, adrenals), magnetic resonance imaging (neck), and bone scan, was performed on all patients.
    Main outcome measures: SRS results were compared with conventional imaging.
    Results: Five of the 11 patients had abnormal findings on SRS. Of the 53 total target lesions present in the patients, only 24.5% were accurately identified through SRS.
    Conclusions: SRS appears to be less sensitive than conventional imaging at detecting the full extent of metastatic disease in children and adolescents with hereditary MTC. SRS incompletely identified sites of tumor and failed to visualize small sites of tumor or liver and lung metastases, and it has a limited role in the evaluation of metastatic disease in pediatric MTC patients.
    MeSH term(s) Adolescent ; Biomarkers, Tumor/analysis ; Biomarkers, Tumor/blood ; Carcinoma, Neuroendocrine ; Child ; Female ; Humans ; Male ; Neoplasm Metastasis ; Octreotide/analogs & derivatives ; Receptors, Somatostatin/metabolism ; Thyroid Neoplasms/blood ; Thyroid Neoplasms/diagnostic imaging ; Thyroid Neoplasms/metabolism ; Thyroid Neoplasms/pathology ; Tomography, Emission-Computed/methods ; Tumor Burden ; Whole Body Imaging
    Chemical Substances Biomarkers, Tumor ; Receptors, Somatostatin ; indium-111-octreotide ; Octreotide (RWM8CCW8GP)
    Language English
    Publishing date 2011-12-07
    Publishing country United States
    Document type Clinical Trial ; Journal Article ; Research Support, N.I.H., Intramural
    ZDB-ID 3029-6
    ISSN 1945-7197 ; 0021-972X
    ISSN (online) 1945-7197
    ISSN 0021-972X
    DOI 10.1210/jc.2011-2766
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