Ultrasonographic features of hip joints in mucopolysaccharidoses type I and II

The primary aim of this study was to assess the ultrasonographic features of hip joints in patients with mucopolysaccharidosis (MPS) type I and II in comparison with healthy population. The secondary aims were to correlate these features with clinical measures and to evaluate the utility of ultrasound in the diagnosis of MPS disease.Sixteen MPS I (n = 3) and II (n = 13) patients were enrolled in the present study and underwent clinical and radiological evaluation, and bilateral high-resolution ultrasonography (US) of hip joints. The distance from the femoral neck to joint capsule (synovial joint space, SJS), joint effusion, synovial hyperthrophy, and local pathological vascularization were evaluated. The results were compared to the healthy population and correlated with clinical and radiological measures.1. There was a difference in US SJS between children with MPS disease and the normative value for healthy population (7mm). Mean values of SJS were 15.81 ± 4.08 cm (right hip joints) and 15.69 ± 4.19 cm (left joints). 2. No inflammatory joint abnormalities were detected in MPS patients. 3. There was a clear correlation between US SJS and patients' age and height, while no clear correlation was observed between SJS and disease severity.1. Patients with MPS I and II present specific features in hip joint ultrasonography. 2. The data suggests that ultrasonography might be effective in the evaluation of hip joint involvement in patients with MPS and might present a valuable tool in facilitating the diagnosis and follow up of the disease

Lipid profile after switching from TDF (tenofovir disoproxil)-containing to TAF (tenofovir alafenamide)-containing regimen in virologically suppressed people living with HIV

Background. Tenofovir disoproxil fumarate (TDF) or its prodrug tenofovir alafenamide fumarate (TAF) are currently being recommended in treatment of HIV infection. Distinct pharmacological properties of these two forms of a this drug make TAF treatment less nephrotoxic and lead to better impact on bone density. Nevertheless, there is a rising concern about possible metabolic adverse effects of TAF. The purpose of this study was to evaluate the effects on the lipid profile among ART (antiretroviral therapy)-experienced patients switching from TDF‑containing to TAF‑containing regimen in the first year after the switch. Methods. Demographic and clinical data of HIV‑positive ART‑experienced patients treated in infectious diseases department was retrospectively collected. Changes of lipid profile with regards to baseline BMI, age and time of ART duration were analyzed. Results. In the group of 36 patients there was a significant increase in total cholesterol levels (+18.43 mg/dl, SD = 23.86 mg/dl, p < 0.0001) and LDL levels (+13.75 mg/dl SD = 23.05 mg/dl, p = 0.001) in first 12 months after switching from TDF‑containing to TAF‑containing regimen. There were no statistically significant changes in both HDL and TG levels observed. Analysis of total cholesterol and LDL levels in certain subpopulations revealed a significant increase within first year after the switch in patients younger than 40 years old and in those whose BMI was within normal range. Conclusions. Presented data suggests that switching from TDF to TAF in ART‑experienced patients may be associated with worsening lipid parameters. Early detection and management of dyslipidemias among HIV‑positive patients are needed

Home enteral nutrition in children—2010 nationwide survey of the polish society for clinical nutrition of children

Published epidemiologic data on the administration rates of enteral/parenteral home nutrition is very limited. The aim of this first nationwide study was to assess the availability of pediatric home enteral nutrition (HEN) services in Poland. The questionnaire was sent to all regional centers providing pediatric HEN services in Poland (n = 14). The analysis included the number of pediatric patients who received HEN in 2010, their demographic characteristics and geographical distribution. Furthermore, the distributions of indications and methods of enteral nutrition administration were analyzed, along with the reasons of withdrawal from the HEN program. The number and fraction of children receiving HEN increased in 2010, from 433 (11.34 per 1 million inhabitants) on January 1st to 525 (13.75) on December 31st. Marked differences were observed in geographical distribution of this parameter, from zero to up to 30 pediatric patients per 1 million inhabitants. Median age of patients was 6 years (range: 9 months–18 years). In most cases, HEN was prescribed due to neurological disorders (n = 337, 64.2%), and administered by means of gastrostomy (n = 450, 85.71%). This study revealed the dynamic development of pediatric HEN services in Poland but also documented their potential regional shortages

Doxycycline in STI Prophylaxis—A Literature Review

Background: Since the implementation of pre-exposure prophylaxis (PrEP) in HIV prevention, a significant increase in the prevalence of other sexually transmitted infections (STIs) has been reported, especially among men who have sex with men (MSM). Doxycycline is being examined as a potential pharmacological agent in preventing these infections. This review aims to summarize available data on the effectiveness and potential side effects of doxycycline for the prevention of bacterial STIs. Methods: We reviewed the National Library of Medicine and the National Center of Biotechnology Information in order to find clinical trials and relevant observational studies regarding doxycycline usage in STI prophylaxis. Results: Doxycycline prophylaxis reduced the risk of acquiring chlamydia, syphilis, and, in a majority of the trials, the risk of gonorrhea. The clinical trials on doxycycline STI prophylaxis were underpowered to determine if doxycycline promotes the selection of resistance in Neisseria gonorrhoeae and Chlamydia trachomatis. Additionally, no serious side effects of this preventive measure have been reported. None of the studies aimed to compare the efficacy of post- and pre-exposure prophylaxis of STDs with doxycycline. Conclusions: The preliminary results regarding STI prophylaxis with doxycycline seem to be promising. Further research is needed to determine the potential risk of doxycycline prophylactic prescription

Evaluation of Clinical Biomarkers Related to CD4 Recovery in HIV-Infected Patients&mdash;5-Year Observation

Human Immunodeficiency Virus infection leads to the impairment of immune system function. Even long-term antiretroviral therapy uncommonly leads to the normalization of CD4 count and CD4:CD8 ratio. The aim of this study was to evaluate possible clinical biomarkers which may be related to CD4 and CD4:CD8 ratio recovery among HIV-infected patients with long-term antiretroviral therapy. The study included 68 HIV-infected patients undergoing sustained antiretroviral treatment for a minimum of 5 years. Clinical biomarkers such as age, gender, advancement of HIV infection, coinfections, comorbidities and applied ART regimens were analyzed in relation to the rates of CD4 and CD4:CD8 increase and normalization rates. The results showed that higher rates of CD4 normalization are associated with younger age (p = 0.034), higher CD4 count (p = 0.034) and starting the therapy during acute HIV infection (p = 0.012). Higher rates of CD4:CD8 ratio normalization are correlated with higher CD4 cell count (p = 0.022), high HIV viral load (p = 0.006) and acute HIV infection (p = 0.013). We did not observe statistically significant differences in CD4 recovery depending on gender, HCV/HBV coinfections, comorbidities and opportunistic infections. The obtained results advocate for current recommendations of introducing antiretroviral therapy as soon as possible, preferably during acute HIV infection, since it increases the chances of sufficient immune reconstruction

Ultrasound images of hip joints.

<p>(Left) Longitudinal scan of hip joint in a 32-year-old patient with an attenuated phenotype of MPS II. (Right) Longitudinal scan of hip joint in a 10-year-old patient with a severe phenotype of MPS II.</p

The results of Pearson’s correlation between values of synovial joint space (right joint capsule) and patients’ age and height.

<p>The results of Pearson’s correlation between values of synovial joint space (right joint capsule) and patients’ age and height.</p

Ultrasound and X-ray images of hip joints.

<p>(Left) Transverse scan of hip joint a 12-year-old patient with a severe phenotype of MPS II. (Right) Radiograph of the pelvis of a 12-year-old patient with a severe phenotype of MPS II: dysostosis multiplex (irregular shape of the pelvis, hypoplastic hip acetabulum, dysplastic hips, osteonecrosis of the femoral heads with flattened acetabula, lopsided head of femur bones).</p

Demographic characteristics of 16 patients with mucopolysaccharidoses.

<p>*Disease classification/severity defined as MPS I—Hurler, Hurler-Scheie, Scheie; MPS II—severe = neuronopathic, attenuated = non-neuronopathic.</p><p>Demographic characteristics of 16 patients with mucopolysaccharidoses.</p