Intravenous immunoglobulin in the management of hemolytic disease of the newborn

Jaundice caused by hemolysis continues to challenge practitioners caring for infants in the NICU. Bilirubin levels can rise quickly in the first days of life, and interventions must be prompt to prevent side effects related to hyperbilirubinemia. Conventional treatments such as hydration and phototherapy are common, but new studies suggest that use of intravenous immunoglobin (IVIG) as an additional treatment may prevent the need for exchange transfusion in some babies. This article presents a case study of an infant with blood-type incompatibility treated successfully with multiple doses of IVIG, discusses the pathophysiology and clinical presentation of hemolytic jaundice, and reviews current management strategies for this disease.     

Sinonasal myxoma: a pediatric case

The authors report a case of a rare facial myxoma arising from the maxillary sinus in a 20-month-old child. The diagnosis was confirmed by a biopsy, and the patient underwent a partial maxillectomy to achieve a total resection of the mass due to the locally aggressive nature of the lesion. Myxomas should be differentiated from malignant sarcomas, in particular embryonal rhabdomyosarcoma, which can arise from the same location and require multimodality therapy consisting of surgery, irradiation, and chemotherapy.     

Validity of maternal report of acute health care use for children younger than 3 years

BACKGROUND: National household surveys often rely on parents' recall to assess children's use of health care services. However, little is known about the accuracy of parental reporting of hospitalizations and emergency department (ED) use. OBJECTIVES: To assess the agreement between maternal reported and medical record acute health care data for children younger than 3 years and to determine if agreement between the 2 varies by maternal characteristics. DESIGN AND METHODS: Data were obtained from the national evaluation of the Healthy Steps for Young Children for 2937 families who completed parent interviews at 2 to 4 and 30 to 33 months and whose children's medical records were abstracted. Services assessed included hospitalizations and ED visits since birth (2-4 and 30-33 months) and in the last 12 months (30-33 months). Absolute and beyond chance agreements were calculated. Results were stratified by maternal age (<20, 20-29, or > or =30 years), parity (first-time, second-time, or greater mother), income (<20,000 dollars, 20,000 dollars-49,999 dollars, or > or =50,000 dollars), and the presence or absence of maternal depressive symptoms. RESULTS: Absolute agreement was high for hospitalizations (> or =90%) at both time points. It was high for ED use (>90%) only at 2 to 4 months. Beyond chance agreement was higher for hospitalizations than for ED use at 2 to 4 and 30 to 33 months. Beyond chance agreement declined with increased duration of recall and younger maternal age. No differences were found by other maternal characteristics. CONCLUSIONS: Mothers have good recall for acute health care events during the first 3 years of their children's lives. This finding suggests that mothers are a good source of information regarding children's acute health care use.     

Benign gynecologic conditions among participants in the Breast Cancer Prevention Trial

OBJECTIVE: This study was undertaken to report on the benign gynecologic conditions occurring among women with an intact uterus at enrollment in the Breast Cancer Prevention Trial of the National Surgical Adjuvant Breast and Bowel Project. STUDY DESIGN: The incidence rates of several benign gynecologic conditions were determined and risks were compared among women receiving tamoxifen and those receiving placebo, based on risk ratios (RRs) with 95% CIs. Comparisons included stratification by menopausal status, body mass index, and history of estrogen use. RESULTS: Compared with women taking placebo, premenopausal women taking tamoxifen had a greater incidence of endometrial polyps (RR = 1.9, 95% CI = 1.55-2.41), leiomyomas (RR = 1.3, 95% CI = 1.14-1.55), endometriosis (RR = 1.9, 95% CI = 1.35-2.70), ovarian cysts (RR = 1.5, 95% CI = 1.20-1.78), and gynecologic surgical procedures, including hysterectomy (RR = 1.6, 95% CI = 1.29-1.88). Postmenopausal women taking tamoxifen also had an increased incidence of endometrial polyps (RR = 2.4, 95% CI = 1.76-3.24), leiomyomas (RR = 1.4, 95% CI = 1.04-1.80), endometriosis (RR = 1.9, 95% CI = 1.29-5.58), and gynecologic surgical procedures, including hysterectomy (RR = 2.2, 95% CI = 1.60-3.13), compared with women taking placebo. All women taking tamoxifen also had an increased incidence of simple endometrial hyperplasia without atypia (overall RR = 2.06, 95% CI = 1.64-2.60) compared with those taking placebo. CONCLUSIONS: Our results strongly support the estrogen agonist role of tamoxifen as the causative factor for the increased risk of endometrial polyps, leiomyomas, endometriosis, and endometrial hyperplasia among women taking this agent.     

Catastrophic antiphospholipid syndrome: atypical presentation in the setting of chronic graft versus host disease: case report and review of the literature

A 44-year-old female developed a catastrophic thrombotic syndrome following allogeneic stem cell transplantation for acute lymphoblastic leukemia. In the context of chronic graft versus host disease, she developed a lethal multi-system thrombotic state as evidenced by stroke, renal failure and cardiac thrombi in association with elevated anticardiolipin antibody. The case is discussed in the framework of the existing literature and derives clinical practice recommendations for this rare but clinically devastating entity.     

Recurrent intractable seizures in children with cortical dysplasia adjacent to dysembryoplastic neuroepithelial tumor

The purpose of this study was to identify the pathologic features that predict postoperative outcome in children with cortical dysplasia adjacent to dysembryoplastic neuroepithelial tumors. We reviewed the records of children with dysembryoplastic neuroepithelial tumor who underwent epilepsy surgery and who had at least 1 year of surgical follow-up. We divided the dysembryoplastic neuroepithelial tumors into three pathology classes (simple, complex, and nonspecific), categorized adjunctive cortical dysplasia into four types, and compared histopathology with seizure outcomes. We identified 26 children with dysembryoplastic neuroepithelial tumors. Dysembryoplastic neuroepithelial tumors were complex in 19 patients (73%), simple in 6 (23%), and nonspecific in 1 (4%). Cortical dysplasia was adjacent to dysembryoplastic neuroepithelial tumors in 18 patients. Six patients had type IA cortical dysplasia, 5 had type IB, 3 had type IIA, and 1 had type IIB. The 3 remaining patients had repeated surgeries; of these, 2 patients had cortical dysplasias of type IA/IB and 1 was type IIA/IIB. Eight (39%) of 18 patients with dysembryoplastic neuroepithelial tumors and cortical dysplasia required further surgery for recurrent intractable seizures (P < .05), whereas none of 8 patients without cortical dysplasia required additional surgery. Of 13 patients with type I cortical dysplasia, only 4 had a poor seizure outcome, whereas all 5 patients with type II had a poor seizure outcome postoperatively (P < .05). Children with dysembryoplastic neuroepithelial tumor and cortical dysplasia often had recurrent intractable seizures postoperatively and required further epilepsy surgery. Cortical dysplasia adjacent to dysembryoplastic neuroepithelial tumor can play a role in the epileptogenicity of dysembryoplastic neuroepithelial tumor. Complete resection of a dysembryoplastic neuroepithelial tumor and its adjacent cortical dysplasia should be considered.