The influence of gonadotropin-releasing hormone agonists on anthropometric change in girls with central precocious puberty

PurposeThe potential effect of gonadotropin-releasing hormone agonist (GnRHa) treatment on the weight of girls with central precocious puberty (CPP) remains a controversy. We investigated anthropometric changes during and after GnRHa treatment among girls with CPP.MethodsThis retrospective study evaluated data from 127 girls with CPP who received GnRHa treatment for ≥2 years. Height, weight, and body mass index (BMI) values were compared at the baseline (visit 1), after 1 year of GnRHa treatment (visit 2), the end of GnRHa treatment (visit 3), and 6–12 months after GnRHa discontinuation (visit 4).ResultsThe height z score for chronological age (CA) increased continuously between visit 1 and visit 4. No significant differences were observed in BMI z score for CA between visits 1 and 4. However, an increasing trend in the BMI z score for bone age (BA) was observed between visits 1 and 4. The numbers of participants who were of normal weight, overweight, and obese were 97, 22, and 8, respectively, at visit 1, compared to 100, 16, and 11, respectively, at visit 4 (P=0.48).ConclusionAmong girls with CPP, the overall BMI z score for CA did not change significantly during or after GnRHa treatment discontinuation, regardless of their BMI status at visit 1. However, the BMI z score for BA showed an increasing trend during GnRHa treatment and a decreasing trend after discontinuation. Therefore, long-term follow-up of BMI changes among girls with CPP is required until they attain adult height

The First Korean Case of Camurati-Engelmann Disease (Progressive Diaphyseal Dysplasia) Confirmed by TGFB1 Gene Mutation Analysis

Camurati-Engelmann disease (CED) is an autosomal dominant progressive diaphyseal dysplasia caused by mutations in the transforming growth factor-β1 (TGFB1) gene. We report the first Korean family with an affected mother and son who were diagnosed with CED. The proband is a 19-yr-old male with a history of abnormal gait since the age of 2. He also suffered from proximal muscle weakness, pain in the extremities, and easy fatigability. Skeletal radiographs of the long bones revealed cortical, periosteal, and endosteal thickenings, predominantly affecting the diaphyses of the upper and lower extremities. No other bony abnormalities were noted in the skull and spine and no remarkable findings were seen on laboratory tests. The patient's mother had a long-standing history of mild limb pain. Under the impression of CED on radiographic studies, we performed mutation analysis. A heterozygous G to A transition at cDNA position +653 in exon 4 of the TGFB1 gene (R218H) was detected in the patient and his mother

The efficacy of immune checkpoint inhibitors in anaplastic lymphoma kinase-positive non-small cell lung cancer

Background Despite recent advances in treating non-small cell lung cancer (NSCLC) with immune checkpoint inhibitors (ICIs), their role in ALK-positive NSCLC patients is unclear. We investigated the efficacy of ICIs in patients with ALK-positive NSCLC. Methods Between 2011 and 2018, a total of 14 ALK-positive NSCLC patients treated with ICIs were evaluated retrospectively. Clinicopathologic features including age, PD-L1 expression, and treatment outcomes were analyzed. RNA expression level and cytolytic activity by ALK positivity were analyzed using The Cancer Genome Atlas (TCGA) and National Cancer Center Research Institute (NCCRI) data sets. Results A total of 13 patients (92.9%) received ALK inhibitors. Patients received a median of three (range 2-8) courses of therapy. The study included nine patients (64.3%) who were PD-L1-high (>50%) and four (28.6%) who were PD-L1-low (<50%). The objective response rate was 14.3% (2/14). The median progression-free survival time was 2.18 months (95% confidence interval [CI] 1.13 months-not reached [NR]). The median overall survival time was 5.67 months (95% CI 3.00 months-NR). RNA expression levels of CD274 were similar between the ALK-positive and negative groups in both TCGA and NCCRI datasets. RNA levels of CD8A in both TCGA and NCCRI data sets were nonsignificantly lower in the ALK-positive group. Cytolytic activity scores including interferon-gamma-related response were lower in the ALK-positive group in the NCCRI but not TCGA dataset. Conclusions Despite high PD-L1-positive rates, ICIs show limited efficacy in ALK-positive NSCLC. Decreased interferon-gamma-related response may underlie these findings.

Expression patterns of influenza virus receptors in the respiratory tracts of four species of poultry

The primary determinant of influenza virus infectivity is the type of linkage between sialic acid and oligosaccharides on the host cells. Hemagglutinin of avian influenza viruses preferentially binds to sialic acids linked to galactose by an α-2,3 linkage whereas hemagglutinin of human influenza viruses binds to sialic acids with an α-2,6 linkage. The distribution patterns of influenza receptors in the avian respiratory tracts are of particular interest because these are important for initial viral attachment, replication, and transmission to other species. In this study, we examined the distribution patterns of influenza receptors in the respiratory tract of chickens, ducks, pheasants, and quails because these species have been known to act as intermediate hosts in interspecies transmission. Lectin histochemistry was performed to detect receptor-bearing cells. Cell-specific distribution of the receptors was determined and expression densities were compared. We observed species-, site-, and cell-specific variations in receptor expression. In general, receptor expression was the highest in quails and lowest in ducks. Pheasants and quails had abundant expression of both types of receptors throughout the respiratory tract. These results indicate that pheasants and quails may play important roles as intermediate hosts for the generation of influenza viruses with pandemic potential

Increased Expression of Sodium Transporters in Rats Chronically Inhibited of Nitric Oxide Synthesis

The present study was done to determine whether endogenous nitric oxide (NO) plays a role in the regulation of sodium transporters in the kidney. Male Sprague-Dawley rats were treated with NG-nitro-L-arginine methyl ester (L-NAME, 100 mg/L drinking water) for 4 weeks. Control rats were supplied with tap water without drugs. Expression of Na, K-ATPase, type 3 Na/H exchanger (NHE3), Na/K/2Cl cotransporter (BSC1), and thiazide-sensitive Na/Cl cotransporter (TSC) proteins was determined in the kidney by Western blot analysis. Catalytic activity of Na,K-ATPase was also determined. The treatment with L-NAME significantly and steadily increased the systemic blood pressure. Total and fractional excretion of urinary sodium decreased significantly, while creatinine clearance remained unaltered. Neither plasma renin activity nor aldosterone concentration was significantly altered. The α1 subunit expression and the catalytic activity of Na, K-ATPase were increased in the kidney. The expression of NHE3, BSC1 and TSC was also increased significantly. These results suggest that endogenously-derived NO exerts a tonic inhibitory effect on the expression of sodium transporters, including Na, K-ATPase, NHE3, BSC1, and TSC, in the kidney

A Case of Primary Neuroendocrine Carcinoma of the Gallbladder Associated with Anomalous Union of the Pancreaticobiliary Duct

Primary neuroendocrine carcinoma of the gallbladder is extremely rare because normal gallbladder mucosa does not contain neuroendocrine cells. Neuroendocrine cells can be detected at sites of intestinal metaplasia induced by chronic inflammation, which may be the initial step in the development of neuroendocrine tumor of the gallbladder. Anomalous union of the pancreaticobiliary duct (AUPBD) is an uncommon congenital anomaly that is frequently associated with choledocholithiasis, cholangitis, pancreatitis, and cancer of the gallbladder or bile duct. In AUPBD, cancers of the gallbladder and bile duct can be induced by chronic inflammation. We report herein a case of large-cell neuroendocrine tumor of the gallbladder associated with AUPBD

Preparation and In Vitro

Magnesium ion substituted biphasic calcium phosphate (Mg-BCP) bioceramic microscaffolds with spherical and porous morphology were successfully prepared using in situ coprecipitation and rotary spray drying atomization process for application of tissue engineering combined with human adipose tissue-derived mesenchymal stem cells (hAT-MSCs). After 4 weeks of immersion in Hanks’ balanced salt solution (HBSS), Mg-BCP micro-scaffolds showed the enhanced biodegradation and bioactivity due to the substituted Mg2+ ion present in the BCP structure. In this study, it was observed that hAT-MSCs have clearly attached on the surface of Mg-BCP micro-scaffolds. In addition, Mg-BCP micro-scaffolds exhibited the improved biocompatibility and osteoconductivity via in vitro and in vivo biological tests with hAT-MSCs. Therefore, these bioceramic micro-scaffolds had potential to be used as hAT-MSCs microcarriers for biomedical applications

Risk Factors for Food Residue after Distal Gastrectomy and a New Effective Preparation for Endoscopy: The Water-Intake Method

Background/Aims: Food residue is frequently ob-served in the gastric remnant after distal gastrectomy, despite adequate preparation. We devised a water-in-take method to reduce food residue in the gastric remnant by drinking large quantities of water in a short time. The aims of this study were to identify the risk factors for food residue and to study the effec-tiveness of this new method for endoscopy prepa-ration. Methods: A cohort of 708 patients who under-went distal gastrectomy for gastric cancer was re-viewed prospectively. Sixty patients with large amo-unts of food residue were randomly divided into two groups: a water-intake group (n=40) and a prolonged fasting group (n=20). Results: The incidences of a large amount of food residue were 15.7%, 5.8%, 7.5%, and 2.8 % at 3, 12, 24, and 36 months, re-spectively, after distal gastrectomy. Independent risk factors for food residue were endoscopy at 3 months, diabetes mellitus, a body mass index of ＜19.5, and laparoscopic surgery. The proportion of successful preparations at follow-up endoscopy was higher for the water-intake group (70%) than for the prolonged fasting group (40%, p=0.025). Conclusions: The wa-ter-intake method can be recommended as a prepara-tion for endoscopy in patients who have had repetitive food residue or risk factors after distal gastrectomy