Abnormal linear growth in paediatric adrenal diseases: Pathogenesis,prevalence and management

Abnormal adrenal function can interfere with linear growth, potentially causing either acceleration or impairment of growth in paediatric patients. These abnormalities can be caused by direct effects of adrenal hormones, particularly glucocorticoids and sex steroids, or be mediated by indirect mechanisms such as the disturbance of the growth hormone-insulin-like growth factor-1 axis and aromatization of androgens to oestrogens. The early diagnosis and optimal treatment of adrenal disorders can prevent or minimize growth disturbance and facilitate improved height gain. Mechanisms of growth disturbance in the following abnormal states will be discussed; hypercortisolaemia, hyperandrogenaemia and obesity. Prevalence and features of growth disturbance will be discussed in ACTH-dependent and ACTH-independent Cushing's syndrome, adrenocortical tumours, premature adrenarche, congenital adrenal hyperplasia and adrenal insufficiency disorders. Recommendations for management have been included.     

Small bowel adenocarcinoma (SBA) three years after colonic adenocarcinoma in an elderly patient: Case report in a National Institute of Health and Aging (INRCA) and review of the literature

INTRODUCTION

Adenocarcinoma of the small intestine is a rare malignancy (the annual incidence in the USA is approximately 3.9 cases per million persons with median age between 60 and 70 years) with limited data available to guide therapeutic decisions. Nonspecific signs and symptoms associated with difficulty in performing small bowel examination is the cause of delayed diagnosis made between 6 and 9 months after appearance of symptoms with the majority of patients presenting with late stage disease and either lymph node involvement or distant metastatic disease.

PRESENTATION OF CASE

An 87-year-old man treated 3 years previously for colonic adenocarcinoma with left colectomy, was brought to our attention with a 4.5 cm × 3.5 cm mass in the proximal jejunum associated with another abdominal wall enhancing mass of 5 cm in diameter in the rectus muscle. Diagnosis on gross examination after surgical resection was adenocarcinoma stage III (T4N1M0) with involvement of lymph nodes.

DISCUSSION

According to an analysis of the Surveillance, Epidemiology and End Results (SEER) database, patients who develop either a small or large intestine adenocarcinoma are at increased risk for a second cancer at both intestinal sites. The role of adjuvant therapy in patients who undergo curative resection is unclear. Recent retrospective and prospective studies have helped to clarify the optimal chemotherapy approach for advanced small bowel adenocarcinoma.

CONCLUSION

With our work, we present our personal case of metachronous primary carcinoma of small bowel following resected colorectal carcinoma and review the literature.     

Hope and spirituality among patients with chronic kidney disease undergoing hemodialysis: a correlational study

Objective

to analyze the relationship between the hope and spirituality of patients with chronic kidney disease undergoing hemodialysis.

Method

this is a cross-sectional, correlational study. The sample was composed of 127 patients of a Renal Replacement Unit. Data were collected through individual interviews guided by the following instruments: participant characterization, Herth Hope Index (HHI), and Pinto Pais-Ribeiro Spirituality Scale (PP-RSS).

Results

the average HHI score was 38.06 (±4.32) while the average PP-RSS score was 3.67 (±0.62) for "beliefs" and 3.21 (±0.53) for "hope/optimism". Spearman''s coefficient indicated there was a moderate positive correlation between the HHI and PP-RSS dimensions of "beliefs" (r=0.430; p<0.001) and "hope/optimism" (r=0.376; p<0.001).

Conclusion

Since a relationship between the sense of hope and spirituality of patients with chronic kidney disease was found, these constructs should be taken into account at the time health professionals deliver care to help patients coping with the disease and treatment.     

Phosphate homeostasis in Bartter syndrome: a case–control study

Background

Bartter patients may be hypercalciuric. Additional abnormalities in the metabolism of calcium, phosphate, and calciotropic hormones have occasionally been reported.

Methods

The metabolism of calcium, phosphate, and calciotropic hormones was investigated in 15 patients with Bartter syndrome and 15 healthy subjects.

Results

Compared to the controls, Bartter patients had significantly reduced plasma phosphate {mean [interquartile range]:1.29 [1.16–1.46] vs. 1.61 [1.54–1.67] mmol/L} and maximal tubular phosphate reabsorption (1.16 [1.00–1.35] vs. 1.41 [1.37–1.47] mmol/L) and significantly increased parathyroid hormone (PTH) level (6.1 [4.5–7.7] vs. 2.8 [2.2–4.4] pmol/L). However, patients and controls did not differ in blood calcium, 25-hydroxyvitamin D, alkaline phosphatase, and osteocalcin levels. In patients, an inverse correlation (P?P?P?Conclusions The results of this study demonstrate a tendency towards renal phosphate wasting and elevated circulating PTH levels in Bartter patients.     

Immune responses of a chimaeric protein vaccine containing Mycoplasma hyopneumoniae antigens and LTB against experimental M. hyopneumoniae infection in pigs

A recombinant chimaeric protein containing three Mycoplasma hyopneumoniae antigens (C-terminal portion of P97, heat shock protein P42, and NrdF) fused to an adjuvant, the B subunit of heat-labile enterotoxin of Escherichia coli (LTB), was used to immunize pigs against enzootic pneumonia. The systemic and local immune responses, as well as the efficacy of the chimaeric protein in inducing protection against experimental M. hyopneumoniae infection were evaluated. In total, 60 male piglets, purchased from a M. hyopneumoniae-free herd, at 4 weeks of age were randomly allocated to six different experimental groups of 10 animals each: recombinant chimaeric protein by intramuscular (IM) (1) or intranasal (IN) (2) administration, commercial bacterin by IM administration (3), and the adjuvant LTB by IM (4, control group A) or IN (5, control group B) administration. All groups were immunized at 24 and 38 days of age and challenged at 52 days of age. The sixth group that was not challenged was used as the negative control (IN [n = 5] or IM [n = 5] administration of the LTB adjuvant). Compared with the non-challenged group, administration of the chimaeric protein induced significant (P < 0.05) IgG and IgA responses against all individual antigens present in the chimaera, but it could not confer a significant protection against M. hyopneumoniae infection in pigs. This lack of effectiveness points towards the need for further studies to improve the efficacy of this subunit-based vaccine approach.     

Heterozygous TREM2 mutations in frontotemporal dementia

A causative association was recently demonstrated between homozygous TREM2 mutations and frontotemporal dementia (FTD)-like syndrome and between heterozygous TREM2 exon2 genetic variations and late-onset Alzheimer's disease (AD). The objective of this study was to evaluate whether heterozygous TREM2 genetic variations might be associated to the risk of FTD. TREM2 exon 2 was sequenced in a group of 1030 subjects—namely, 352 patients fulfilling clinical criteria for FTD, 484 healthy control subjects (HCs), and 194 patients with AD. The mutation frequency and the associated clinical characteristics were analyzed. We identified 8 missense and nonsense mutations in TREM2 exon 2 in 24 subjects. These mutations were more frequent in patients with FTD than in HCs (4.0% vs. 1.0%, p = 0.005). In particular, TREM2 Q33X, R47H, T66M, and S116C mutations were found in FTD and were absent in HCs. These mutations were associated with either the semantic variant of primary progressive aphasia or the behavioral variant FTD phenotypes. The FTD and AD groups were not significantly different with regard to TREM2 genetic variation frequency (AD: 2.6%, p = 0.39). Heterozygous TREM2 mutations modulate the risk of FTD in addition to increasing susceptibility to AD. Additional studies are warranted to investigate the possible role of these mutations in the pathogenesis of neurodegenerative disorders.     

The response of some blood constituents after administration of two different diets in goats

Twenty clinically healthy goats were divided into two equal groups based on two different diets. Goats in group A were fed with 25 % barley, 12 % oat, 15 % faba bean, 10 % pea, 20 % sugar beet pulps, 5 % molasses and 3 % mineral. Goats in group B were fed with 25 % corn, 15 % soybean hulls, 12 % soybean meal, 10 % sunflower meal, 20 % sugar beet pulps, 5 % molasses and 3 % mineral. To establish the effect of diet composition on haematological and biochemical concentrations, blood samples were collected from the external jugular vein once a week, for 1 month (T0–T4), twice daily (0700 and 1800 hours). Multivariate analysis of variance (MANOVA) showed a significant effect of different diets (P?P?P?相似文献