Urinary tract infections in a neonatal intensive care unit.

OBJECTIVE: Our aim was to describe laboratory findings and imaging results for neonatal patients diagnosed with urinary tract infection (UTI). STUDY DESIGN: Medical records were reviewed for infants diagnosed with UTI in a single neonatal intensive care unit (NICU) over a 13-year period. RESULTS: Of the 8,241 patients admitted to the NICU during the study period, 137 infants were diagnosed with UTI. Imaging was reviewed for 101 patients. Renal pelvis dilation was found in 34% of patients and vesicoureteral reflux was found in 21%. Renal pelvis dilation was not associated with reflux (OR: 0.53 [95% CI: 0.18-1.5]). The sensitivity of urinalysis to detect a positive culture was 76%, and the specificity was 41%. Tests of cure for bacterial infections were uniformly negative. CONCLUSION: Renal pelvis dilation was common but not associated with reflux among NICU patients diagnosed with UTI. Diagnostic criteria in this population are not well defined, and guidelines are needed for diagnosis and management of UTIs in NICU patients.

Automated pediatric abdominal effective diameter measurements versus age-predicted body size for normalization of CT dose.

There has been increasing interest in adjusting CT radiation dose data for patient body size. A method for automated computation of the abdominal effective diameter of a patient from a CT image has previously only been tested in adult patients. In this work, we tested the method on a set of 128 pediatric patients aged 0.8 to 12.9 years (average 8.0 years, SD = 3.7 years) who had CT abdomen/pelvis exams performed on a Toshiba Aquilion 64 scanner. For this set of patients, age-predicted abdominal effective diameter extrapolated based on data from the International Commission on Radiation Units and Measurements was a relatively poor predictor of measured effective diameter. The mean absolute percentage error between the CTDI normalization coefficient calculated from a manually measured effective diameter and the coefficient determined by age-predicted effective diameter was 12.3 % with respect to a 32 cm phantom (range 0.0-52.8 %, SD 8.7 %) and 12.9 % with respect to a 16 cm phantom (range 0.0-56.4 %, SD 9.2 %). In contrast, there is a close correspondence between the automated and manually measured patient effective diameters, with a mean absolute error of 0.6 cm (error range 0.2-1.3 cm). This correspondence translates into a high degree of correspondence between normalization coefficients determined by automated and manual measurements; the mean absolute percentage error was 2.1 % with respect to a 32 cm phantom (range 0.0-8.1 %, SD = 1.4 %) and 2.3 % with respect to a 16 cm phantom (range 0.0-9.3 %, SD = 1.6 %).

Large Mesenteric Gaucheroma Responds to Substrate Reduction Therapy: A New Management of Gaucheromas.

Gaucheromas, which are pseudotumors consisting of a cluster of Gaucher cells, are rare complications in Gaucher's disease (GD) and reported in patients treated with enzyme replacement therapy (ERT). Gaucheromas commonly develop in the lymph nodes in the mesenteric and mediastinal regions and can cause serious complications including protein-losing enteropathy. A large mesenteric Gaucheroma showed a significant reduction in size after initiation of substrate reduction therapy (SRT) with eliglustat in an adult patient with GD type 3. Combination therapy with ERT and SRT should be considered to prevent Gaucheromas in patients with GD.

Sclerosing bone dysplasias: review and differentiation from other causes of osteosclerosis.

Sclerosing bone dysplasias are skeletal abnormalities of varying severity with a wide range of radiologic, clinical, and genetic features. Hereditary sclerosing bone dysplasias result from some disturbance in the pathways involved in osteoblast or osteoclast regulation, leading to abnormal accumulation of bone. Several genes have been discovered that, when disrupted, result in specific types of hereditary sclerosing bone dysplasia (osteopetrosis, pyknodysostosis, osteopoikilosis, osteopathia striata, progressive diaphyseal dysplasia, hereditary multiple diaphyseal sclerosis, hyperostosis corticalis generalisata), many of which exhibit similar pathologic mechanisms involving endochondral or intramembranous ossification and some of which share similar underlying genetic defects. Nonhereditary dysplasias include intramedullary osteosclerosis, melorheostosis, and overlap syndromes, whereas acquired syndromes with increased bone density, which may simulate sclerosing bone dysplasias, include osteoblastic metastases, Paget disease of bone, Erdheim-Chester disease, myelofibrosis, and sickle cell disease. Knowledge of the radiologic appearances, distribution, and associated clinical findings of hereditary and nonhereditary sclerosing bone dysplasias and acquired syndromes with increased bone density is crucial for accurate diagnosis.

Racial differences in growth patterns of children assessed on the basis of bone age.

PURPOSE: To collect up-to-date data in healthy children to create a digital hand atlas (DHA) that can be used to evaluate, on the basis of the Greulich and Pyle atlas method, racial differences in skeletal growth patterns of Asian, African American, white, and Hispanic children in the United States. MATERIALS AND METHODS: This retrospective study was HIPAA compliant and approved by the institutional review board. Informed consent was obtained from all subjects or their guardians. From May 1997 to March 2008, a DHA containing 1390 hand and wrist radiographs obtained in male and female Asian, African American, white, and Hispanic children with normal skeletal development was developed. The age of subjects ranged from 1 day to 18 years. Each image was read by two pediatric radiologists working independently and without knowledge of the subject's chronologic age, and evaluation was based on their experience with the Greulich and Pyle atlas. Statistical analyses were performed with the paired-samples t test and analysis of variance to study racial differences in growth patterns. P

Testicular microlithiasis in patients with Down syndrome.

OBJECTIVES: Testicular microlithiasis (TM) occurs with benign as well as with pathological conditions, such as testicular cancer. Since Down syndrome (DS) may be associated with increased frequency of testicular cancer, we determined the prevalence of TM in DS in patients from a DS clinic and evaluated the prevalence by age group. STUDY DESIGN: We compared results of research scrotal ultrasounds obtained from 1998 to 2001 from 92 Latino patients with DS (ages newborn to 29.7 years) and clinical ultrasounds obtained from 1998 to 2004 from 200 Latino patients without DS (ages newborn to 18.3 years). We also reviewed the medical records. RESULTS: The prevalence of TM in DS was 29%, significantly higher than the 7% found in patients without DS (P < .0001). Twenty of the 27 patients with DS and TM had no testicular pathology clinically or by history. The TM prevalence in the entire group of patients with and without DS increased with advancing age. CONCLUSIONS: We found a significantly increased prevalence of TM in DS. The clinical significance of TM needs to be investigated further.