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  1. Article ; Online: Autoimmune Liver Disease in Patients With Sickle Cell Disease.

    Waisbourd-Zinman, Orith / Frenklak, Rachel / Hakakian, Odelia / Hilmara, Didja / Lin, Henry

    Journal of pediatric hematology/oncology

    2021  Volume 43, Issue 7, Page(s) 254–257

    Abstract: Objective: Hepatic and biliary tract diseases are common in sickle cell disease (SCD) patients, likely due to sickling, hemosiderosis, viral hepatitis, or cholelithiasis. Literature is lacking on associations between SCD, autoimmune hepatitis (AIH), and/ ...

    Abstract Objective: Hepatic and biliary tract diseases are common in sickle cell disease (SCD) patients, likely due to sickling, hemosiderosis, viral hepatitis, or cholelithiasis. Literature is lacking on associations between SCD, autoimmune hepatitis (AIH), and/or sclerosing cholangitis (SC)-together, autoimmune liver disease (AILD). We aimed to better understand the relationship of these diseases in pediatric patients.
    Materials and methods: A retrospective analysis of patients with SCD and AILD at the Children's Hospital of Philadelphia (January 2008 to August 2015).
    Results: Seven patients, ages 8 to 23 years (3 males), were identified. Three had AIH, 2 SC, and 2 AIH/SC overlap, known as autoimmune SC. All patients with AIH treated with azathioprine significantly improved their liver enzymes. One patient with SC and inflammatory bowel disease underwent successful bone marrow transplant. Two SC patients died from SCD complications.
    Conclusions: In this cohort, there seems to be an association between SCD and AILD; SC in this population was severe. Physicians should be aware of this and evaluate patients with SCD and elevated liver enzymes for AILD.
    MeSH term(s) Adolescent ; Adult ; Anemia, Sickle Cell/complications ; Child ; Erythrocytes, Abnormal/pathology ; Female ; Follow-Up Studies ; Hepatitis, Autoimmune/etiology ; Hepatitis, Autoimmune/pathology ; Humans ; Male ; Prognosis ; Retrospective Studies ; Young Adult
    Language English
    Publishing date 2021-02-11
    Publishing country United States
    Document type Journal Article
    ZDB-ID 1231152-2
    ISSN 1536-3678 ; 1077-4114 ; 0192-8562
    ISSN (online) 1536-3678
    ISSN 1077-4114 ; 0192-8562
    DOI 10.1097/MPH.0000000000001985
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 1537: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

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  2. Article ; Online: Pregnancy and Childbirth After Spinal Fusion for Adolescent Idiopathic Scoliosis.

    Landrum, Matthew / Nocka, Heidi R / Ashebo, Leta / Hilmara, Didja / MacAlpine, Elle / Flynn, John M / Ho, Michelle / Newton, Peter O / Sponseller, Paul D / Lonner, Baron S / Cahill, Patrick J

    Journal of pediatric orthopedics

    2023  Volume 43, Issue 10, Page(s) 620–625

    Abstract: Background: Little data exist on pregnancy and childbirth for adolescent idiopathic scoliosis (AIS) patients treated with a spinal fusion. The current literature relies on data from patients treated with spinal fusion techniques and instrumentation, ... ...

    Abstract Background: Little data exist on pregnancy and childbirth for adolescent idiopathic scoliosis (AIS) patients treated with a spinal fusion. The current literature relies on data from patients treated with spinal fusion techniques and instrumentation, such as Harrington rods, that are no longer in use. The objective of our study is to understand the effects of spinal fusion in adolescence on pregnancy and childbirth.
    Methods: Prospectively collected data of AIS patients undergoing posterior spinal fusion that were enrolled in a multicenter study who have had a pregnancy and childbirth were reviewed. Results were summarized using descriptive statistics and compared with national averages using χ 2 test of independence.
    Results: A total of 78 babies were born to 53 AIS patients. As part of their pre-natal care, 24% of patients surveyed reported meeting with an anesthesiologist before delivery. The most common types of delivery were spontaneous vaginal delivery (46%, n=36/78) and planned cesarean section (20%, n=16/78). Compared with the national average, study patients had a higher rate of cesarean delivery ( P =0.021). Of the women who had a spontaneous vaginal birth, 53% had no anesthesia (n=19/36), 19% received intravenous intermittent opioids (n=7/36), and 31% had regional spinal or epidural anesthesia (n=11/36). spontaneous vaginal delivery patients in our study cohort received epidural or spinal anesthesia less frequently than the national average ( P <0.001). Of those (n=26 pregnancies) who did not have regional anesthesia (patients who had no anesthesia or utilized IV intermittent opioids), 19% (n=5 pregnancies) were told by their perinatal providers that it was precluded by previous spine surgery.
    Conclusion: The majority of AIS patients reported not meeting with an anesthesiologist before giving birth and those who had a planned C-section did so under obstetrician recommendation. The presence of instrumentation after spinal fusion should be avoided with attempted access to the spinal canal but should not dictate a delivery plan. A multidisciplinary team consisting of obstetrician, anesthesiologist, and orthopaedic surgeon can provide the most comprehensive information to empower a patient to make her decisions regarding birth experience anesthesia based on maternal rather than provider preference.
    Level of evidence: IV.
    Language English
    Publishing date 2023-08-22
    Publishing country United States
    Document type Journal Article
    ZDB-ID 604642-3
    ISSN 1539-2570 ; 0271-6798
    ISSN (online) 1539-2570
    ISSN 0271-6798
    DOI 10.1097/BPO.0000000000002499
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.A 1681: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

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  3. Article ; Online: Steroid Free Treatment of Autoimmune Hepatitis in Selected Children.

    Wehrman, Andrew / Waisbourd-Zinman, Orith / Shah, Amit / Hilmara, Didja / Lin, Henry / Rand, Elizabeth B

    The Journal of pediatrics

    2019  Volume 207, Page(s) 244–247

    Abstract: Current guidelines recommend steroids for induction of remission in all children diagnosed with autoimmune hepatitis regardless of the clinical presentation. In this report, we describe our experience in treating selected asymptomatic children with ... ...

    Abstract Current guidelines recommend steroids for induction of remission in all children diagnosed with autoimmune hepatitis regardless of the clinical presentation. In this report, we describe our experience in treating selected asymptomatic children with autoimmune hepatitis using a steroid-free regimen; this treatment strategy was safe and effective in inducing remission.
    MeSH term(s) Adolescent ; Alanine Transaminase/blood ; Antibodies, Antineutrophil Cytoplasmic/blood ; Azathioprine/therapeutic use ; Biomarkers/blood ; Biopsy ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Glucocorticoids/pharmacology ; Hepatitis, Autoimmune/blood ; Hepatitis, Autoimmune/diagnosis ; Hepatitis, Autoimmune/therapy ; Humans ; Liver/pathology ; Male ; Prednisolone/analogs & derivatives ; Prednisolone/pharmacology ; Remission Induction/methods ; Retrospective Studies ; Treatment Outcome
    Chemical Substances Antibodies, Antineutrophil Cytoplasmic ; Biomarkers ; Glucocorticoids ; Prednisolone (9PHQ9Y1OLM) ; Alanine Transaminase (EC 2.6.1.2) ; prednylidene (IF8PQP966U) ; Azathioprine (MRK240IY2L)
    Language English
    Publishing date 2019-02-02
    Publishing country United States
    Document type Comparative Study ; Journal Article ; Research Support, N.I.H., Extramural
    ZDB-ID 3102-1
    ISSN 1097-6833 ; 0022-3476
    ISSN (online) 1097-6833
    ISSN 0022-3476
    DOI 10.1016/j.jpeds.2018.12.070
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Um IV Zs.116: Show issues Location:
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    ab Jg. 2022: Lesesaal (EG)

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  4. Article ; Online: Assessment of caregiver expectations of physician communication in a pediatric setting.

    Lee, Tyler / Cui, Julie / Rosario, Hinette / Hilmara, Didja / Samuelson, Kate / Lin, Emery C / Miller, Victoria A / Lin, Henry C

    BMC health services research

    2020  Volume 20, Issue 1, Page(s) 408

    Abstract: Background: In pediatrics, communication often occurs through an intermediary such as a caregiver. The goal of this study is to assess caregiver communication expectations and determine if meeting expectations influences caregiver satisfaction or ... ...

    Abstract Background: In pediatrics, communication often occurs through an intermediary such as a caregiver. The goal of this study is to assess caregiver communication expectations and determine if meeting expectations influences caregiver satisfaction or instruction retention.
    Methods: A survey study was performed at the Children's Hospital of Philadelphia. Before the visit, caregivers completed a survey on communication expectations, Caregiver Expected Kalamazoo Essential Elements Communication Checklist (Caregiver Expected KEECC). After the visit, caregivers were surveyed on their perception of physician communication (Caregiver Perceived KEECC) and satisfaction. Caregivers were contacted 1 week after the clinic visit to assess instruction retention. Meeting of caregiver expectation was calculated by the difference between Caregiver Expected and Caregiver Perceived KEECC scores.
    Results: 112 caregivers participated in the study. There was no significant difference in Caregiver Expected KEECC versus Caregiver Perceived KEECC score (4.39 vs 4.56). Caregiver communication expectations were exceeded in 51.5% of the visits. Communication expectations were exceeded more among caregivers with at a college education (p <  0.01) and more among White caregivers (p <  0.01). The average caregiver satisfaction score with the clinic visit was 4.67. Higher satisfaction scores were observed in caregivers who had their communication expectations met or exceeded (p <  0.01). Caregivers with communication expectations exceeded had higher percentage recall of physician instructions (p <  0.01).
    Conclusions: Caregiver communication expectations may be influenced by demographic factors. Communication expectation affects visit outcomes including caregiver satisfaction and instruction retention. Therefore, physicians need to be cognizant of caregiver communication expectations, which can impact quality of the healthcare experience.
    MeSH term(s) Ambulatory Care ; Caregivers/psychology ; Checklist ; Child ; Communication ; Delivery of Health Care ; Female ; Hospitals, Pediatric ; Humans ; Male ; Motivation ; Patient Satisfaction ; Pediatrics ; Philadelphia ; Physicians ; Pilot Projects ; Professional-Family Relations ; Surveys and Questionnaires
    Language English
    Publishing date 2020-05-11
    Publishing country England
    Document type Journal Article
    ZDB-ID 2050434-2
    ISSN 1472-6963 ; 1472-6963
    ISSN (online) 1472-6963
    ISSN 1472-6963
    DOI 10.1186/s12913-020-05262-x
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: Correlation of Pulmonary Function to Novel Radiographic Parameters of Collapsing Parasol Deformity in Spinal Muscular Atrophy.

    Johnson, Mitchell A / Galagedera, Nirupa / Ho, Susanne / Hilmara, Didja / Campbell, Robert M / Anari, Jason B / Mayer, Oscar H / Cahill, Patrick J

    Orthopedics

    2020  Volume 44, Issue 2, Page(s) e287–e293

    Abstract: Spinal muscular atrophy (SMA) is a neuromuscular disease with manifestations of scoliosis, pulmonary function decline, and, uniquely, collapse of the ribs. Methods to quantify rib deformity and its impact on pulmonary function are sparse. The authors ... ...

    Abstract Spinal muscular atrophy (SMA) is a neuromuscular disease with manifestations of scoliosis, pulmonary function decline, and, uniquely, collapse of the ribs. Methods to quantify rib deformity and its impact on pulmonary function are sparse. The authors propose new radiographic measurements to quantify the aspect of SMA known as collapsing parasol deformity and correlate these measurements with pulmonary function. Twenty-eight full-spine radiographs of pediatric SMA patients were measured twice by 3 independent investigators, with 2 weeks separating each measurement. Radiographic measurements, demographics, spirometry results, and assisted ventilation rating were obtained. Twenty-one patients with spirometry metrics were assessed to correlate pulmonary function and spinal measurements. The intrarater intraclass correlation coefficient (ICC) for the measurements ranged from 0.706 to 0.99, and the interrater ICC ranged from 0.64 to 0.97. Eighteen of 19 variables had ICC values greater than 0.75 for inter- and intrarater reliability. Twenty-one patients with forced expiratory volume in 1 second and forced vital capacity were assessed in terms of these measurements. Ratio of the concave hemithoracic width at T6/convex hemithoracic width at T6 (
    MeSH term(s) Adolescent ; Child ; Humans ; Lung/diagnostic imaging ; Lung/physiopathology ; Male ; Muscular Atrophy, Spinal/diagnostic imaging ; Muscular Atrophy, Spinal/physiopathology ; Muscular Atrophy, Spinal/surgery ; Orthopedic Procedures ; Radiography ; Reproducibility of Results ; Ribs/physiopathology ; Ribs/surgery
    Language English
    Publishing date 2020-12-30
    Publishing country United States
    Document type Journal Article
    ZDB-ID 424447-3
    ISSN 1938-2367 ; 0147-7447
    ISSN (online) 1938-2367
    ISSN 0147-7447
    DOI 10.3928/01477447-20201216-05
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 1487: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

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