Pediatric Primary Health Care: The Central Role of Pediatricians in Maintaining Children's Health in Evolving Health Care Models.

Pediatric primary health care (PPHC) is of principal importance to the health and development of all children, helping them reach their true potential. Pediatricians, as the clinicians most intensively trained and experienced in child health, are the natural leaders of PPHC within the context of the medical home. Given the rapidly evolving models of pediatric health care delivery, including the explosion of telehealth in the wake of the COVID-19 pandemic, pediatricians, together with their representative national organizations such as the American Academy of Pediatrics (AAP), are the most capable clinicians to guide policy innovations on both the local and national stage.

Congenital amegakaryocytic thrombocytopenia in three siblings: molecular analysis of atypical clinical presentation.

Objective: An 11-year-old girl, presenting with fatigue and bruising, was found to be profoundly pancytopenic. Bone marrow exam and clinical evaluation were consistent with aplastic anemia. Family members were studied as potential stem cell donors, revealing that both younger siblings displayed significant thrombocytopenia, whereas both parents had normal blood counts.

Survival after recurrence of Ewing's sarcoma family of tumors.

Purpose: The overall survival (OS) of patients with relapsed Ewing's sarcoma family of tumors (ESFT) is poor, and the relative benefit of high-dose therapy (HDT) is controversial.

Patients And Methods: We retrospectively identified 55 consecutive ESFT patients with adequate medical records for review, who were treated at Children's Hospital and Regional Medical Center and who developed disease recurrence between January 1, 1985 and December 31, 2002.

Results: The median relapse-free interval (RFI) from diagnosis to first recurrence was 17 months (range, 5 to 90 months).

Prescription medication use during pregnancy and risk of infant leukemia (United States).

Objective: We explored whether maternal medication use during pregnancy may be an important etiologic area for investigation in studies of infant leukemia.

Methods: In this case-control study, associations were explored between specific medications as recorded in the medical records of 243 mothers of infants who were diagnosed with leukemia at < 18 months of age and 393 mothers ol infants without leukemia identified through random digit dialing. Cases included 157 acute lymphoblastic (ALL), 77 acute myeloid (AML), and nine other leukemias.

Tumors and malformations of the caudal spinal axis.

The early development of the neural tube has been well studied in animals and humans. After axial determinants have been accomplished the processes of primary and secondary neurulation take place. Successful completion results in a spinal cord that has arisen from primary neurulation and a lower sacro-coccygeal portion from secondary neurulation.

Pregnancy outcome of partners of male survivors of childhood cancer: a report from the Childhood Cancer Survivor Study.

Purpose: This study was undertaken to determine the effect, if any, on pregnancy loss, live births, and birthweight of treatment for cancer diagnosed during childhood or adolescence.

Patients And Methods: We reviewed pregnancy outcome among sexually active male Childhood Cancer Survivor Study (CCSS) participants who responded to a questionnaire before February 3, 2000. Medical records of all members of the cohort were abstracted to obtain chemotherapeutic agents administered, the cumulative dose of drug administered for several drugs of interest, and the doses, volumes, and dates of administration of all radiotherapy.

Pregnancy outcome of female survivors of childhood cancer: a report from the Childhood Cancer Survivor Study.

Objective: This study was undertaken to determine the effect, if any, of prior treatment with radiation therapy or chemotherapy for cancer diagnosed during childhood or adolescence on pregnancy loss, live births, and birth weight.

Study Design: We reviewed pregnancy outcome among female participants in the Childhood Cancer Survivor Study (CCSS) who returned a questionnaire. Eligibility for the CCSS included 5-year survivors who were <21 years old at diagnosis and who were diagnosed with an eligible cancer between January 1, 1970, and December 31, 1986, at the 25 participating CCSS institutions.

Peripheral blood stem cell support reduces the toxicity of intensive chemotherapy for children and adolescents with metastatic sarcomas.

Background: To increase the dose intensity (DI) of chemotherapy for pediatric patients with metastatic sarcomas, including the Ewing sarcoma family of tumors (ESFT) and rhabdomyosarcoma (RMS), the authors tested the feasibility of an intensive regimen supported by granulocyte-colony stimulating factor (G-CSF) and peripheral blood stem cells (PBSC).

Methods: Twenty-three children and adolescents with metastatic sarcomas received vincristine, doxorubicin, cyclophosphamide, ifosfamide, sodium mercaptoethanesulfonate (mensa), and etoposide (VACIME) chemotherapy, consisting of 8 courses of vincristine 2 mg/m(2) on Day 0, doxorubicin 37.5 mg/m(2) per day on Days 0-1, cyclophosphamide 360 mg/m(2) per day on Days 0-4, ifosfamide 1800 mg/m(2) per day on Days 0-4, mesna 2400 mg/m(2) per day, and etoposide 100 mg/m(2) per day on Days 0-4.

Sex ratios in congenital malformations of the central nervous system.

Malformations of the central nervous system (CNS) are evaluated and treated by pediatric neurosurgeons. There is a spectrum of morphological variations within each type of malformation and with associated features that may determine whether the defect is isolated or part of a syndrome. Alterations in the normal sex ratio have been found in many malformations of the CNS but the reasons for them are usually unclear.