Retrospective evaluation of the orthodontic treatment needs in primary school children with Robin sequence following Tübingen palatal plate therapy in infancy.

INTRODUCTION: The aim of this study was to investigate the orthodontic treatment needs (OTN) of children with RS treated with the TPP in infancy compared to age- and sex-matched controls. METHODS: The aim of this study was to investigate the orthodontic treatment needs (OTN) of children with RS treated with the TPP in infancy compared to age- and sex-matched controls. RESULTS: In 21 children with RS (n = 23; 19 non-syndromic, 4 syndromic; average age 9.9 years) showed high OTN, which was significantly higher than in controls (n = 21). The latter of 9 controls had minor OTN, followed by 8 participants with borderline OTN. Regarding the intraoral picture, patients with RS had an increased open bite tendency. Without considering the presence of a cleft palate, 16 children with RS had high or very high OTN, compared to 4 of controls. CONCLUSIONS: Patients with RS have significantly higher OTN than healthy controls, independent of cleft occurrence. RS is associated with dental anomalies and special skeletal growth patterns, both increasing malocclusion and negatively affecting dentoalveolar growth. This should raise awareness for identifying these needs and provide a comprehensive orthodontic treatment, where functional rehabilitation should be favored over aesthetic results.

Arsenic-containing hydrocarbons: effects on gene expression, epigenetics, and biotransformation in HepG2 cells.

Arsenic-containing hydrocarbons (AsHCs), a subgroup of arsenolipids found in fish and algae, elicit substantial toxic effects in various human cell lines and have a considerable impact on cellular energy levels. The underlying mode of action, however, is still unknown. The present study analyzes the effects of two AsHCs (AsHC 332 and AsHC 360) on the expression of 44 genes covering DNA repair, stress response, cell death, autophagy, and epigenetics via RT-qPCR in human liver (HepG2) cells. Both AsHCs affected the gene expression, but to different extents. After treatment with AsHC 360, flap structure-specific endonuclease 1 (FEN1) as well as xeroderma pigmentosum group A complementing protein (XPA) and (cytosine-5)-methyltransferase 3A (DNMT3A) showed time- and concentration-dependent alterations in gene expression, thereby indicating an impact on genomic stability. In the subsequent analysis of epigenetic markers, within 72 h, neither AsHC 332 nor AsHC 360 showed an impact on the global DNA methylation level, whereas incubation with AsHC 360 increased the global DNA hydroxymethylation level. Analysis of cell extracts and cell media by HPLC-mass spectrometry revealed that both AsHCs were considerably biotransformed. The identified metabolites include not only the respective thioxo-analogs of the two AsHCs, but also several arsenic-containing fatty acids and fatty alcohols, contributing to our knowledge of biotransformation mechanisms of arsenolipids.

Improvement of sickle cell anemia by iron-limited erythropoiesis.

We report the hematologic and clinical features of four adult patients (Pts.) with sickle cell anemia and iron-limited erythropoiesis. Two of the Pts. had spontaneous iron deficiency (chronic GI bleeding, low-grade hemoglobinuria). In the other two Pts. iron restriction was induced by periodic RBC aphereses as part of a pilot protocol designed to decrease intracellular HbS polymerization by MCHC reduction. Iron-limited erythropoiesis was defined by reduction in red cell indices (MCV range 60.4-67 fl) in the presence of low serum ferritin (range < 10-20 ng/ml). In these Pts. iron restriction did not cause clinically significant worsening of the anemia (Hb 7.8-9.0 g/dl). In two Pts. the anemia actually improved. Other hematologic effects of iron restriction were: decreased MCHC, reticulocyte count, RDW, and dense cells. A reduced hemolytic rate was suggested by a lowering of serum bilirubin and LDH. In one of the Pts. the 51Cr RBC T1/2 survival increased from 12 to 16 days. The intracellular HbS polymer fractions (fp) were determined at 25% O2 by Csat and with the use of the conservation of mass equation. The baseline fp values ranged from 0.48-0.53. After iron restriction they ranged from 0.33-0.48. The fp decreased even though iron-limited erythropoiesis also lowered the Hb F concentration in three of our Pts. In one of the two Pts. with induced iron depletion, hospitalization days for pain crises decreased from an average of 4.5 days/month (2 year baseline period) to an average of 0.5 days/month in the 3 year follow-up after iron depletion. The second patient with induced iron restriction experienced the rapid healing of a leg ulcer. Controlled iron restriction should be explored as a therapeutic strategy in selected SS patients.

Enhancement of pain control with ketorolac tromethamine in patients with sickle cell vaso-occlusive crisis.

Twenty one patients with sickle cell disease admitted to the hospital with the pain of vaso-occlusive crisis (VOC) were treated by continuous IV infusion of ketorolac or normal saline for up to 5 days. All patients received supplemental IM injections of meperidine, 100 mg, as necessary, but not more frequently than every 3 hr. Over the 5 days the ketorolac treated patients (KT) required 33% less meperidine than did the placebo treated patients (PL), P = 0.04, and had significantly better pain relief as assessed by categorical, visual analog, and pain relief scales. By the end of 5 days infusions had been discontinued in six KT and one PL. The time to discontinuation of the infusion was significantly shorter in KT, (P = 0.009). The median duration of hospital stay from the start of treatment was 3.3 days for KT and 7.2 days for PL, P = 0.027. Adverse events were mainly related to the digestive system. This study showed that continuous infusion of ketorolac significantly reduced total meperidine requirement and that the analgesia produced by this combination was superior to that produced by meperidine alone. Further evaluation of this drug in the management of sickle cell VOC is warranted.

Patient-controlled analgesia in patients with sickle cell vaso-occlusive crisis.

Pain control using intramuscular analgesia is often unsatisfactory in sickle cell patients. In a pilot study, 15 patients with sickle cell anemia (SS) and one patient with SB thalassemia in vaso-occlusive crisis were treated with the Patient-Controlled Analgesia (PCA) technique using a Pharmacia Deltec Programmable pump (CADD PCA). Age range was 19-50 years (median = 27); there were nine females and seven males. The protocol consisted of 3 days of therapy using a background of continuous infusion meperidine. The starting dose was 20 mg/hr and was escalated to 30 mg/hr. The average amount given was 25.8 mg/hr. One to two boluses of 2.5-5.0 mg/dose (mode = 5.0) were also allowed each hour. In addition, patients number 8 through 16 were given hydroxyzine (Vistaril) 50 mg PO q6h. The number of days in pain prior to study entry (mean +/- SD) was 3.3 +/- 1.6. The number of pain sites per patient was 3.6 +/- 1.2. Using categorical and analog pain scales, patients' pain scores decreased only about 30%. However, most patients were fairly satisfied with the treatment and rated it overall as follows: 1 poor, 1 fair, 3 good, 6 very good, 4 excellent, 1 no comment. Patients number 8 through 16 gave higher ratings probably because a more idealized dosage regimen was being used by that time in the study. There were no adverse effects or major problems noted. It is our impression that PCA, when optimized, will be a safe and effective alternative method for providing patients with sickle cell vaso-occlusive crisis pain relief.

[A contribution to the stabilisation of the MVA-vaccine (author's transl)]. / Ein Beitrag zur Stabilisierung des MVA-Impfstoffs.

The titre of the MVA-vaccine remains constant at -20 degrees C with human serum albumin, sorbitol, Haemaccel, Rheomacrodex and cysteine within 8 month. At -20 degrees C the stability of the MVA-virus is equivalent to the Vaccinia-virus, stabilized by peptone and sorbitol. Storing the MVA-Virus at +4 degrees C, +20 degrees C for 8 month or at 27 degrees C for 4 weeks, 1% human serum albumin and 5% sorbitol preserves the virustitre best, but the stability of Vaccinia-virus with 5% peptone and 5% sorbitol can, however, not be obtained by the MVA-virus under these conditions.