Nutrient deficiencies in children with celiac disease during long term follow-up.

BACKGROUND: Celiac disease (CD) is associated with malabsorption and consequential nutritional deficiencies. Patients with CD must follow a gluten-free diet (GFD), which is also associated with nutrient deficiencies. Despite the clinical significance, consensus is lacking on the pattern and frequency of nutrient deficiencies in CD and the usefulness of assessment during follow-up. The aim was to investigate the presence of micronutrient and protein deficiencies in pediatric patients with CD after starting a GFD and receiving standard clinical care, taking disease activity into account. METHODS: This single center retrospective chart review aimed to map the occurrence of nutrient deficiencies, determined in serum samples derived during follow-up in an expertise center for pediatric CD. Serological micronutrient levels were determined during routine clinical visits up until 10 years in children with CD on a GFD. RESULTS: The data of 130 children with CD was included. When pooling the measurements between 3 months and 10 years after GFD initiation, a deficiency in iron, ferritin, vitamin D, vitamin B12, folate and zinc was present in 33%, 21,9%, 21,1%, 2,4%, 4,3% and 8,1% of measurements, respectively. No hypocalcemia or vitamin B6 deficiency was found. CONCLUSION: The prevalence of deficiency varies amongst nutrients in children following a GFD, a high prevalence of some nutrient deficiencies is noteworthy. This study highlights the necessity to structurally investigate the risk of developing nutrient deficiencies while following a GFD. Knowledge of the risk to develop deficiencies can contribute to achieving a more evidence based approach in the management and follow-up of CD in children.

Microfluidics for single-cell genetic analysis.

The ability to correlate single-cell genetic information to cellular phenotypes will provide the kind of detailed insight into human physiology and disease pathways that is not possible to infer from bulk cell analysis. Microfluidic technologies are attractive for single-cell manipulation due to precise handling and low risk of contamination. Additionally, microfluidic single-cell techniques can allow for high-throughput and detailed genetic analyses that increase accuracy and decrease reagent cost compared to bulk techniques. Incorporating these microfluidic platforms into research and clinical laboratory workflows can fill an unmet need in biology, delivering the highly accurate, highly informative data necessary to develop new therapies and monitor patient outcomes. In this perspective, we describe the current and potential future uses of microfluidics at all stages of single-cell genetic analysis, including cell enrichment and capture, single-cell compartmentalization and manipulation, and detection and analyses.

Primary bone lymphoma in a 10-year-old boy.

Primary bone lymphoma has been defined as a solitary lesion in bone, without concomitant involvement of the extra osseous hematopoietic system, with no evidence of extra osseous disease within 6 months of the onset of symptoms. The vast majority of cases are of the large B-cell non-Hodgkin type. They are rare bone tumor. Distinguishing primary bone lymphoma from other bone tumors is important because the former has a better response to therapy and a better prognosis.

Imaging of superior labral anterior to posterior (SLAP) tears of the shoulder.

Superior labral anterior to posterior (SLAP) tears include a number of abnormal changes of the superior glenoid labrum. SLAP tears have been first reported in elite young atlete and are caused by repetitive overhead motion or by a fall on an outstretched arm. SLAP can lead to chronic pain and instability of shoulder. A diagnosis of SLAP may be difficult on the basis of clinical tests. Hence, modern imaging, including computed tomography arthrography (CTA), magnetic resonance imaging (MRI) and magnetic resonance arthrography (MRA) play a key role in the diagnosis of SLAP. The large number of normal anatomic variants of the superior labrum and the surrounding structures make the interpretation of SLAP challenging on imaging and at arthroscopy. In this article the imaging of SLAP are discussed in detail along with relevant anatomy, anatomic variants and biomechanics.

Intracranial germ cell tumor.

Germ cell tumours represent about 3 to 8% of pediatric brain tumours. Occurrence of diabetes insipidus is common in the case of suprasellar germ cell tumors. The diagnosis may be advanced by MRI owing to the location and relatively univocal characteristics of the lesion signal. The existence of a bifocal mass developed in both suprasellar region and pineal zone is highly suggestive of a germinoma. The most important notion is to recognize that at the time of diabetes insipidus diagnosis in a child, the cerebral mass might be too small to be identified by MRI. In such patients, repeating imaging study should be obtained.

Percutaneous penetration/dermal absorption of hair dyes in vitro.

This paper presents an in vitro technique to analyse percutaneous penetration and dermal absorption of hair dyes, topically applied to excised pig skin. Representative examples are given by the radio-labelled hair dyes p-phenylenediamine and bis-(5-amino-1-hydroxyphenyl)-methane. Both compounds were assessed under simulated use conditions and were analysed in representative formulations including the specific conditions for oxidation hair dyes. To be able to differentiate between topically adsorbed and systemically available amounts, the bioavailability of the hair dyes is defined as the amount penetrated and/or remaining in the exposed skin after removing the stratum corneum. Less than 1% of the assessed topically applied dyes was found to be bioavailable in the presence of hydrogen peroxide, typically added to oxidation hair dyes prior to applications. Compared with published results and unpublished in-house in vivo data, the level of confidence was high. Owing to in-house experience over about 5 years in using excised pig skin for measurements of percutaneous penetration and dermal absorption of hair dyes, the technique was found to be successful and appropriate to reduce the number of test animals normally used for toxicological assessments. The essentials of this technique are actually recommended by the SCCNFP (The Scientific Committee on Cosmetic Products and Non Food Products intended for Consumers) for the safety evaluation of cosmetic ingredients, particularly for hair dyes. The corresponding OECD guideline as well as the guidance document has been drafted and is currently in discussion on the level of the national coordinators.

Regional anesthesia in pediatric surgery: complications and postoperative comfort level in 174 children.

Postoperative pain control (PPC) in children is a difficult management problem. Systemic narcotics often result in respiratory depression, while nonnarcotic analgesics are associated with inconsistent PPC. This report reviews a 29-month (January 1989 through July 1991) experience with 174 children (aged < 18 years) who received regional PPC through indwelling catheters. There were 105 males and 69 females. Patient age ranged from 1 day to 17 years 10 months (mean age, 97 months). All catheters were placed using introduction needles ranging from 24 to 16 gauge. Agents were delivered as either continuous infusion (151 patients, 87%) or bolus injections (23 patients, 13%). Analgesics were age- and weight-determined dosages of bupivacaine with or without narcotic supplementation. All patients had surgical procedures except two who had catheters placed for pain control after trauma and one who had a catheter for intractable abdominal pain of unknown etiology. Twenty-five (15%) children had thoracic incisions, 76 (43%) abdominal, 16 (9%) flank, and 54 (31%) extremity. Catheter placement included 40 thoracic epidurals (23%), 100 lumbar (57%), 27 caudal (16%), and 7 pleural (4%). Catheters were utilized for a duration of 0.5 to 8 days (mean, 2.1 +/- 1.2 days). One hundred forty-four children required no additional pain medications (83%). Thirty (17%) patients required supplemental medications. Acetaminophen was used in 6 (3%), acetaminophen with codeine in 4(2%), morphine in 18 (10%), and Percocet in 1(1%). Minor complications occurred 21 times in 16 children (9%).(ABSTRACT TRUNCATED AT 250 WORDS)

Improved outcome utilizing spinal anesthesia in high-risk infants.

The development of apnea following general anesthesia in high-risk infants (less than 60 weeks postconceptual age) has been reported up to 37%, prompting the routine admission of these children following minor surgical procedures. One hundred forty high-risk infants (American Society of Anesthesiologists category greater than or equal to 2) were prospectively evaluated after undergoing surgical procedures normally performed as outpatients in low-risk babies. All patients had spinal anesthesia for their operations. The mean gestational age for these infants was 30.8 +/- 3.7 weeks (minimum, 24 weeks) with a mean birth weight of 1,466.0 +/- 638.8 g. The mean postconceptual age and weight at the time of surgery were 44.8 +/- 7.8 weeks and 3,336 +/- 1,242 g, respectively. Difficulty in administering the spinal anesthetic occurred in 6 cases (4.2%). Postoperative complications occurred in 5 children (3.8%). They were: postoperative fever (2), transient bradycardia (2), and apnea (1). The four cases of postoperative fever and bradycardia were insignificant and required no medical intervention. The single case of apnea occurred in a premature infant who received a supplemental dose of intravenous midazolam. Length of operation in these cases ranged from 15 minutes to 95 minutes (mean, 53 minutes), with two incidents of inadequate anesthesia occurring in this cohort. Mean duration of anesthesia was 146 minutes (range, 50 to 240 minutes) and was directly dependent on dosage administration of the agents. These data indicate that the use of spinal anesthesia in high-risk infants is safe and effective for surgical procedures generally performed as outpatients (3.0% minor complication rate, 0.8% major complication rate).(ABSTRACT TRUNCATED AT 250 WORDS)

Levy-Hollister syndrome.

The Levy-Hollister syndrome is an autosomal dominant disorder characterized by lacrimal malformations, simple cup-shaped ears, hearing loss, hypodontia and enamel dysplasia, and upper limb malformations. Renal anomalies have been noted variably. Two families with this disorder have been described previously. Recently, a third family with the Levy-Hollister syndrome was evaluated. Unusual features present in this family included bilateral nasolacrimal duct fistulas, radial aplasia, and unusual dermal ridge patterns. Early recognition of this disorder should prompt investigation for renal anomalies and/or hearing loss. It should also lead to consideration of surgical attempts to correct the lacrimal abnormalities or conductive hearing loss, thereby reducing the long-term morbidity in affected patients.