BackgroundWe evaluated the effectiveness of FDG‐PET scans in identifying sites of active disease and assessing response to therapy in patients with Langerhans cell histiocytosis (LCH). Changes in standardized uptake value (SUV) indicated increased or decreased disease activity before changes are evident by plain films or bone scans.MethodsOne hundred and two PET scans for 44 patients (41 children, 3 adults) with biopsy‐proven LCH were compared with 83 corollary imaging modalities and were rated for overall clinical utility: false positive or negative (“inferior”), confirming lesions identified by another imaging modality (“confirmatory”), or showing additional lesions, response to therapy or recurrence of disease activity (“superior”), in comparison to bone scans, MRI, CT or plain films.ResultsFDG‐PET was rated superior in that 90/256 (35%) new, recurrent, or lesions responding to therapy were identified via change in standard uptake value (SUV) before other radiographic changes. PET scans confirmed active LCH in 146/256 (57%). FDG‐PET was superior to bone scans in that 8/23 (34%) lesions, 11/53 (21%) comparisons to lesions found by MRI, 13/64 (20%) CT, and 58/116 (50%) plain films. PET scans confirmed lesions found by: 14/23 (61%) bone scans, 33/53 (62%) MRI, 45/64 (65%) CT, and 54/116 (46%) of plain films.ConclusionsWhole body FDG‐PET scans can detect LCH activity and early response to therapy with greater accuracy than other imaging modalities in patients with LCH lesions in the bones and soft tissues. Whole‐body FDG‐PET scanning is an important and informative study at diagnosis and for following disease course in patients with LCH. Pediatr Blood Cancer 2009;52:97–101. © 2008 Wiley‐Liss, Inc.

A unique specimen of unilateral Sprengel's deformity, and contralateral normal pectoral girdle, were studied morphologically and roentgenographically. The cervical spine exhibited multiple abnormalities involving both the vertebral centra as well as the posterior elements (Klippel-Feil abnormality). A small spina bifida involving C5 and C6 was present. Abnormalities of the spinous processes included an articulation with a well-formed omovertebral bone that also articulated with the vertebral (infraspinatus) margin of the scapula. Secondary (presumed epiphyseal) ossification was present in the omovertebral bone at the distal end. The scapula was deformed, especially in the supraspinatus portion. The clavicle was shorter and had a different contour.

African Americans, testing for at least the 25 recommended mutations. Results: A total of 33 CF carriers were identified. The most common mutations detected were ⌬F508, G622D, R117H/7T, and G551D. The G622D allele frequency among African Americans was 0.18%. We did not detect any 3120ϩ1G3 A carriers, although 4 were expected (P Ͻ 0.05). Conclusions: When considering only the 25 recommended CF mutations, 1 in 75 African Americans screened in our laboratories were carriers (within the expected range, given a 69% mutation detection rate). The addition of 2 mutations, G622D and Q98R (incidentally

OBJECTIVE: Rates of maternal morbidity in the US are accelerating partly due to increasing comorbidities among pregnant women. Proposals to adopt levels of maternal care (LOMC), similar to neonatal care, would direct patients to riskappropriate care earlier in pregnancy, theoretically reducing maternal transports, optimizing resources, and improving outcomes. The objective of this study is to describe characteristics and indications for maternal transport by LOMC within a state transport system. STUDY DESIGN: This is a retrospective cohort study of all maternal transports from Sept '15 e Jun '19 to Johns Hopkins Hospital (JHH, one of two Level IV centers within Maryland) or JH Bayview (JHB, Level III). Data were extracted from transport logs and electronic medical records; transports for neonatal indication were excluded. Continuous variables were compared using ANOVA, categorical variables using Chi-square, and a p-value < 0.05 used for statistical significance. RESULTS: 652 transports for maternal indication were recorded. Transport indication varied significantly by LOMC (P< 0.001): hospitals with 'no L&D' most commonly transported for

Most patients with brain and skull base tumors can travel safely via commercial airflight with acceptable symptom exacerbation. However, consideration should be given to administering corticosteroids and possibly anticonvulsants to patients who are symptomatic and/or have relatively large tumors with mass effect and peritumoral edema.

Minimal residual disease may predict bone marrow relapse in patients with hairy cell leukemia treated with 2-chlorodeoxyadenosine. Blood, 87, 1556-1560.

Doxorubicin (DOX) is a potent, commonly used anthracycline antibiotic for a wide range of cancers, but its dose is limited by its strong cardiotoxic effects. The glucose analog 2-deoxy-D-glucose (2-DG) has been shown to enhance the antitumor efficacy of DOX in tumor cell lines and in animals. However, the cardiac effects of 2-DG in these experiments were not determined. In the current study, we examined whether 2-DG could protect from DOX cardiotoxicity in a BALB/c mouse breast cancer model. A total of 32 mice were injected with 10 5 4T1 mouse mammary epithelial tumor cells in the right hind leg. The tumor was allowed to progress for 1 week before dividing the mice into four groups: saline, 2-DG, DOX, and 2-DG with DOX treatment. 2-DG was given 150 mg/kg i.p. Monday through Friday; DOX was given 5 mg/kg i.p. Wednesday. The study was conducted for 3 weeks and echocardiography was performed the day before sacrifice. Two of the eight mice in the DOX group died before echocardiography, while none died in the 2-DG with DOX group. Echocardiography indicated similar cardiac function between saline and 2-DG groups as measured by fractional shortening (36.3±1.31% vs 37.7±0.942%, p>0.05). DOX markedly reduced cardiac function, which was almost completely recovered by 2-DG (25.1±1.06% vs 36.7±1.76%, p<0.01). Serum CK-MB content, a common clinical marker for heart damage, was increased ~6 fold in the DOX group (67.5±19.4 vs 400±145, p<0.023). 2-DG tended to reduce DOX-triggered CK-MB release, but it did not reach significant levels likely due to the small sample size and high variation (279±68.7 vs 400±145, p>0.05). Nonetheless, 2-DG largely eliminated DOX-induced apoptosis in the heart as shown by a DNA laddering assay. 2-DG or DOX alone increased cardiac autophagic flux as measured by the difference in LC3-II protein levels in the absence and presence of the lysosomal inhibitor, bafilomycin A1. Paradoxically, autophagic flux was not notably elevated when mice were treated with 2-DG and DOX simultaneously. These results demonstrate the ability of 2-DG to reduce DOX cardiotoxicity without affecting its antitumor activity, suggesting that 2-DG can improve the therapeutic window for DOX, allowing greater flexibility in designing different regimens for treating cancers.

INTRODUCTION: Implementing levels of maternal care (LOMC) dictates that certain maternal conditions be centralized to risk-appropriate care centers. However, the feasibility of a LOMC system is dependent on the ability to transport risk appropriate patients in a timely manner. The objective of this study is to describe maternal transports via a robust, state-sponsored maternal transport system. METHODS: This is a retrospective cohort study of all maternal transports from Sept ‘15 through Jun ‘19 into the Johns Hopkins Medical system. Data were extracted from maternal transport logs and electronic medical records. RESULTS: Of 1,392 maternal transports over the study period, 652 (47%) were for maternal indications. The majority of transports originated from Level I centers (37%). The median distance travelled per transport was 26 miles: 70% by ambulance and 21% by helicopter. 48% of transported mothers delivered during their admission at the receiving hospital. Average maternal age was 29 years, with 48% of mothers being white, 42% black. Average infant weight was 1926 grams, with a 66% NICU admission rate. 18% of transports were suspected preventable if antepartum care was transferred prior to the delivery episode with the most frequent indications for transport being hypertensive disease of pregnancy (34%), infection (8%) and placentation (6%). CONCLUSION: Maryland has had an established, regionalized maternal care system since the early-1990s, with clearly defined LOMC and a functioning transport network. Our findings demonstrate utilization patterns within such a program and describe key characteristics of transporting hospitals and patients. Further research is needed to determine the effectiveness of LOMC.