Early treatment of intrapericardial teratoma: a case presentation and systematic literature review

Objective: To present a case of an early treatment of cardiac intraperitoneal teratoma (IPT) in a newborn and its associated systematic literature review.

Methods: We presented a case of a newborn with IPT but without hydrops and having a good perinatal outcome after cardiac surgery. Using the PubMed database, we conducted a systematic literature review of articles regarding cases with cardiac IPT diagnosed and treated in the neonatal period and published in English from 2004 onward. We excluded cases that involved fetal death or interrupted gestation events.

Results: In total, 38 cases of IPT from 31 articles were included. The mean?±?standard deviation of the gestational age at diagnosis and delivery were 27.9?±?5.7 and 33.0?±?3.5 weeks, respectively, and that of birth weight was 2373?±?834.5?g. The majority of fetuses (42.1%) were males. Pericardial effusion was the most common symptom (60.5%) followed by hydrops (42.1%) and respiratory distress (42.1%). Intrauterine procedure was not performed in 63.1% of cases, and 71.0% of newborns were alive.

Conclusions: IPT in newborns is usually associated with a good prognosis without the need for intrauterine procedures. Cases with IPT-related death are associated with hemodynamic impairment in fetuses with hydrops.     

Optimal care for the child with cancer: A summary statement from the SIOP working committee on psychosocial issues in pediatric oncology

Since its foundation in 1991, the SIOP Working Committee on Psychosocial Issues in Paediatric Oncology 1 has developed and published 12 sets of Guidelines for health‐care professionals treating children with cancer and their families. Those elements considered essential in the process of cure and care of children with cancer are summarized in this document as a formal statement, developed at the 2007 SIOP annual meeting in Mumbai. Elaboration of the concepts with detailed strategies for practice can be found in the referenced guidelines [1–12] and in a companion publication [13]. This article is a summary of what practitioners considered critical elements in the optimal care of the child with cancer, with the goal of stimulating a broader application of these elements throughout the SIOP membership. Pediatr Blood Cancer 2009;52:904–907. © 2009 Wiley‐Liss, Inc.     

Interferon treatment for chronic hepatitis B: enhanced response in children 5 years old or younger

OBJECTIVE: To test the hypothesis that there is an improved response to interferon in children with chronic hepatitis B (HBV) who are < or =5 years of age. STUDY DESIGN: Retrospective chart review of 22 consecutive children with chronic HBV (ages 17 months to 17 years; median, 83.9 months; 14 male, 8 female) treated with interferon-alpha2b. RESULTS: Ten patients (48%) responded to treatment [HBeAg (-), Anti-HBe (+), HBV DNA (-), HBsAg (+) and normal alanine aminotransferase/aspartate aminotransferase (ALT/AST) at 6 months after treatment], and 5 seroconverted HBsAg [above plus HBsAg negative and anti-HBs (+)]. Seven of 9 patients (78%) < or =5 years of age responded (5 cleared HBsAg). Three of 13 patients (23%) >5 years of age responded. Patient age at treatment was significantly lower in responders (63 +/- 70 months) versus nonresponders (104 +/- 55 months, P =.005). AST, ALT, and HBV DNA at the start of treatment were not different between responders and nonresponders or between patients < or =5 and >5 years old. CONCLUSIONS: Interferon treatment may be more effective in younger children with chronic hepatitis B.     

Combining the Creative Arts and the House Call to Teach Medical Students About Chronic Illness Care

As the number of people living with chronic illness in the United States rises, it is imperative that medical school prepare physicians who are capable of caring for these patients. This article outlines a creative educational intervention to teach third‐ and fourth‐year medical students about caring for chronically ill people. All students at Weill Medical College, Cornell University, make home visits to homebound older adults with an interdisciplinary team as part of a mandatory Primary Care Clerkship. Under their guidance, students observe the myriad challenges facing homebound older adults. Afterward, students create a project, using original or found art, to express their reactions and thoughts. Students present projects to peers in a small group, with multidisciplinary faculty mentors framing the discussion. To evaluate the intervention, students responded to a nine‐item questionnaire and a series of open‐ended questions. Quantitative and qualitative analyses show consistently strong positive responses to the experience. Most students (95.0%) felt that they learned about the complexities of chronic illness care from their home visits. The opportunity to express reactions through a creative project received positive responses with 97.0% of students responding favorably. Ninety‐seven percent felt that the discussions with colleagues and faculty increased knowledge of chronic illness care. Nearly all (97.0%) felt they had a better understanding of team and that attitudes toward the chronically ill were positively affected. The coupling of the creative arts with home visits is an effective tool for teaching about chronic illness and may be a useful model for medical schools interested in expanding their chronic illness curriculum.     

Surgical anatomy of the midfoot

The midfoot plays a vital role in the stability of the foot as a whole. Injuries to the midfoot may be subtle and can lead to significant long-term morbidity, especially where there is a delay in diagnosis and treatment. This article describes the anatomy of the midfoot that is relevant to surgical practice. It should provide the reader with an understanding of how the anatomy of the midfoot relates to its function as well as discussing the anatomical considerations of injuries and surgery in this area. Anatomical knowledge aids clinical practice in terms of the recognition of abnormalities and the guidance of treatment.     

Isolated systolic hypertension,obesity, and hyperkinetic hemodynamic states in children

OBJECTIVE: To determine the factors that contribute to the pathogenesis of isolated systolic hypertension in children. METHODS: School-based measurement was performed of blood pressure (BP), heart rate, weight, and height in 2460 students (49% Hispanic, 31% black, 13% white) 12 to 16 years of age in 8 urban public schools. An independent group of 71 untreated children underwent 24-hour ambulatory BP monitoring (ABPM) to confirm clinic hypertension and assess circadian BP patterns. RESULTS: Hypertension and obesity were found in 17% and 23% of students, respectively. Among hypertensive students, 88% (363/413) had isolated systolic hypertension. Hypertension was more prevalent in obese than nonobese students (33% vs 11%, P <.0001). Obese hypertensive students had higher resting heart rate than nonobese normotensive patients (85.9 vs 79.6 beats/min, P <.001). Among patients who underwent ABPM, isolated systolic hypertension was found in 51% (36/71) by clinic BP and in 62% (18/29) with confirmed hypertension by ABPM. Blood pressure variability during daytime and sleep periods was higher in obese than nonobese patients for systolic BP (P <.01) and diastolic BP (P <.05). CONCLUSIONS: The findings of increased heart rate and BP variability in obese children with isolated systolic hypertension suggest that sympathetic nervous system hyperactivity may contribute to its pathogenesis.     

Niemann-pick disease type C in neonatal cholestasis at a North American Center

OBJECTIVE: To determine the frequency of Niemann-Pick disease type C (NPC) among children being evaluated for neonatal cholestasis during a 2-year period. METHODS: Medical records were reviewed from all infants with cholestasis and all patients with NPC evaluated at our center from January 1997 through December 1998. RESULTS: Forty neonates with cholestasis were evaluated, including three patients diagnosed with NPC (age at diagnosis, 5-21 months) who were originally labeled as having idiopathic neonatal cholestasis (INH). Two adolescents (ages 14 and 16 years) were also diagnosed with NPC during this period, one who originally had neonatal hepatitis and cirrhosis, and the other who had hepatosplenomegaly throughout childhood. Three of the patients with NPC were Hispanic. At time of NPC diagnosis, infants had mildly delayed motor development and persistent splenomegaly with or without hepatomegaly, and the adolescents had ataxia, dysarthria, hepatosplenomegaly, and paresis of vertical gaze. The diagnosis of NPC was established by demonstrating defective cellular cholesterol esterification in cultured skin fibroblasts in three patients and a specific genetic mutation in three patients. Niemann-Pick disease type C was found in 27% of infants initially diagnosed with INH and 8% of all infants evaluated for cholestasis. CONCLUSION: Niemann-Pick disease type C should be considered in all infants with cholestasis, particularly those with splenomegaly or who are of Hispanic descent. Electron microscopy and lipid analysis of liver biopsy specimens obtained during the evaluation of neonatal cholestasis may suggest this diagnosis.     

Human hepatic flavin-containing monooxygenases 1 (FMO1) and 3 (FMO3) developmental expression.

The flavin-containing monooxygenases (FMOs) are important for the metabolism of numerous therapeutics and toxicants. Six mammalian FMO genes (FMO1-6) have been identified, each exhibiting developmental and tissue- and species-specific expression patterns. Previous studies demonstrated that human hepatic FMO1 is restricted to the fetus whereas FMO3 is the major adult isoform. These studies failed to describe temporal expression patterns, the precise timing of the FMO1/FMO3 switch, or potential control mechanisms. To address these questions, FMO1 and FMO3 were quantified in microsomal fractions from 240 human liver samples representing ages from 8 wk gestation to 18 y using Western blotting. FMO1 expression was highest in the embryo (8-15 wk gestation; 7.8 +/- 5.3 pmol/mg protein). Low levels of FMO3 expression also were detectable in the embryo, but not in the fetus. FMO1 suppression occurred within 3 d postpartum in a process tightly coupled to birth, but not gestational age. The onset of FMO3 expression was highly variable, with most individuals failing to express this isoform during the neonatal period. FMO3 was detectable in most individuals by 1-2 y of age and was expressed at intermediate levels until 11 y (12.7 +/- 8.0 pmol/mg protein). These data suggest that birth is necessary, but not sufficient for the onset of FMO3 expression. A gender-independent increase in FMO3 expression was observed from 11 to 18 y of age (26.9 +/- 8.6 pmol/mg protein). Finally, 2- to 20-fold interindividual variation in FMO1 and FMO3 protein levels were observed, depending on the age bracket.