Nonketotic hyperglycinemia in two siblings with neonatal seizures

Seizures are a common problem in neonates. Differential diagnoses include infection, trauma, hypoxia and congenital metabolic disorders. Among these, congenital metabolic disorder is less familiar to general pediatricians. We report two patients with nonketotic hyperglycinemia (NKH), a rare and lethal congenital metabolic disease. Transient hyperammonemia and transient hypouricemia, uncommon features found in NKH, were detected in one patient. High doses of sodium benzoate and dextromethorphan failed to modify the clinical course. Neuropathology denoted characteristic diffuse vacuolization and changes in reactive and gliotic astrocytes. The clinical course, biochemical findings, diagnostic approaches and diagnostic tests are discussed in detail. Recent modalities of treatment are reviewed. Because of its rarity and rapidly progressive course, it maybe underdiagnosed resulting in death before being recognized. Awareness of the possibility of congenital metabolic disorder in early neonatal catastrophe will increase the diagnostic rate.     

Afatinib as First-line Treatment of Older Patients With EGFR Mutation-Positive Non-Small-Cell Lung Cancer: Subgroup Analyses of the LUX-Lung 3, LUX-Lung 6, and LUX-Lung 7 Trials

Background

Afatinib is approved in the US, Europe, and several other regions for first-line treatment for epidermal growth factor receptor mutation-positive (EGFRm⁺) non-small-cell lung cancer (NSCLC).

Management of Seborrhea and Enlarged Pore Size with a Hydrogel Containing Ficus Fistulosa Extract

BackgroundTannins are considered an important phytochemical used in cosmetics and cosmeceuticals and are well-known for their astringent and antioxidant properties. Ficus fistulosa is a plant that grows in the Southern part of Thailand. Its fruits have an astringent taste, which might correlate with the presence of tannins.ObjectiveWe sought to evaluate the efficacy of a hydrogel containing F. fistulosa extract for controlling sebum.MethodsFirst, the extract of F. fistulosa fruit was evaluated for its tannin content and astringent activity in vitro. Then it was formulated into a hydrogel in different concentrations. After, the safety and sebum-control efficacy of this hydrogel was tested in 10 healthy volunteers. The safety of this hydrogel was evaluated in volunteers using a closed patch test.ResultsThe results of the first part of the study indicated that F. fistulosa extract contained 0.0359±0.0003mg of tannic acid equivalent per gram of extract, and its astringent activity was 87.45%±1.76%. When formulated into hydrogels of different concentrations, the results showed that the hydrogel containing 0.1% w/w had the highest stability profile. No irritation or allergy was observed. Separately, after usage twice per day for 28 consecutive days, the sebum content of the volunteers was decreased by −54.36%±13.71% relative to baseline.ConclusionThe results from this study suggest that F. fistulosa fruit extract might be an effective active ingredient for use by the cosmetic and cosmeceutical industry.     

A bedside prediction-scoring model for late-onset neonatal sepsis.

OBJECTIVE: Insufficient tools for bedside prediction of late-onset neonatal sepsis (LNS) initiated this study. The objective was to develop and validate a simple prediction-scoring model for LNS defined as culture-proven sepsis occurring 72 hours after birth. METHODS: The study was performed at a university hospital in Bangkok. The derivation phase included medical records of 1870 neonates, randomly selected from 9347 records of neonates who had been hospitalized for >72 hours during 1998 to 2000, of which 1824 records were available. In all, 100 neonates were clinically suspected of sepsis and 17 had proven LNS. The validation phase included 73 neonates suspected of having sepsis during July 2002 to June 2003 and 25 who had LNS. Weighted coefficients from Cox's proportional hazards model and receiver-operating-characteristic (ROC) curve analysis were used. RESULTS: The incidence density of LNS was 17/11355 (1.5/1000) person-days. A scoring model was developed and consisted of the following: hypotension (score 4), abnormal body temperature (score 3), respiratory insufficiency (score 2), neutrophil band form fraction >1% (score 2), platelet count <150 x 10(3)/microl (score 2), and umbilical venous catheterization (1 to 7 or >7 days; score 2 or 4). The area under the ROC curves for prediction of LNS in a neonate suspected of sepsis in each of the two phases was 0.85 and 0.80, respectively (p=0.436). The mean probabilities of LNS were approximately 0.10 (low risk) for scores from 0 to 3; 0.50 (intermediate risk) for scores from 4 to 6; and 0.70 (high risk) for scores > or =7. CONCLUSION: A simple prediction-scoring model for LNS was developed. Validation of the scores suggested good diagnostic performance.     

Changes of anterior maxillary alveolar bone thickness following incisor proclination and extrusion

Objective:To investigate changes in maxillary alveolar bone thickness after maxillary incisor proclination and extrusion during anterior crossbite correction in a group of growing patients with Class III malocclusion.Materials and Methods:Maxillary incisors of 15 growing patients with anterior crossbite were proclined and extruded with 0.016″ beta-titanium advancing loops and Class III elastics. Lateral cephalograms were recorded before advancement (T₀) and 4 months after a normal overjet and overbite were achieved (T₁). Changes in alveolar bone thickness surrounding the maxillary incisors at the crestal (S1), midroot (S2), and apical (S3) levels were measured using cone-beam computed tomography (CBCT). Paired t-tests were used to determine the significance of the changes. A Spearman rank correlation analysis was performed to explore the relationship between thickness changes and the rate and amount of incisor movements.Results:Although statistically significant decreases were observed in palatal and total bone thickness at the S2 and S3 level (P < .05), the amounts of these changes were clinically insignificant, ranging from 0.34 to 0.59 mm. Changes in labial bone thickness at all levels were not significant. Changes in palatal bone thickness at S3 were negatively correlated with changes in incisor inclination. (r  =  −0.71; P < .05).Conclusion:In a group of growing patients with Class III malocclusion undergoing anterior crossbite correction, controlled tipping mechanics accompanied by extrusive force may produce successful tooth movement with minimal iatrogenic detriment to the alveolar bone.     

Quantification of lean bodyweight

BACKGROUND: Lean bodyweight (LBW) has been recommended for scaling drug doses. However, the current methods for predicting LBW are inconsistent at extremes of size and could be misleading with respect to interpreting weight-based regimens. OBJECTIVE: The objective of the present study was to develop a semi-mechanistic model to predict fat-free mass (FFM) from subject characteristics in a population that includes extremes of size. FFM is considered to closely approximate LBW. There are several reference methods for assessing FFM, whereas there are no reference standards for LBW. PATIENTS AND METHODS: A total of 373 patients (168 male, 205 female) were included in the study. These data arose from two populations. Population A (index dataset) contained anthropometric characteristics, FFM estimated by dual-energy x-ray absorptiometry (DXA - a reference method) and bioelectrical impedance analysis (BIA) data. Population B (test dataset) contained the same anthropometric measures and FFM data as population A, but excluded BIA data. The patients in population A had a wide range of age (18-82 years), bodyweight (40.7-216.5 kg) and BMI values (17.1-69.9 kg/m2). Patients in population B had BMI values of 18.7-38.4 kg/m2. A two-stage semi-mechanistic model to predict FFM was developed from the demographics from population A. For stage 1 a model was developed to predict impedance and for stage 2 a model that incorporated predicted impedance was used to predict FFM. These two models were combined to provide an overall model to predict FFM from patient characteristics. The developed model for FFM was externally evaluated by predicting into population B. RESULTS: The semi-mechanistic model to predict impedance incorporated sex, height and bodyweight. The developed model provides a good predictor of impedance for both males and females (r2 = 0.78, mean error [ME] = 2.30 x 10(-3), root mean square error [RMSE] = 51.56 [approximately 10% of mean]). The final model for FFM incorporated sex, height and bodyweight. The developed model for FFM provided good predictive performance for both males and females (r2 = 0.93, ME = -0.77, RMSE = 3.33 [approximately 6% of mean]). In addition, the model accurately predicted the FFM of subjects in population B (r2 = 0.85, ME = -0.04, RMSE = 4.39 [approximately 7% of mean]). CONCLUSIONS: A semi-mechanistic model has been developed to predict FFM (and therefore LBW) from easily accessible patient characteristics. This model has been prospectively evaluated and shown to have good predictive performance.