Primary lymphedema is a rare chronic pathology associated with constitutional abnormalities of the lymphatic system. The objective of this French National Diagnosis and Care Protocol (Protocole National de Diagnostic et de Soins; PNDS), based on a critical literature review and multidisciplinary expert consensus, is to provide health professionals with an explanation of the optimal management and care of patients with primary lymphedema. This PNDS, written by consultants at the French National Referral Center for Primary Lymphedema, was published in 2019 (https://has-sante.fr/upload/docs/application/pdf/2019-02/pnds_lymphoedeme_primaire_final_has.pdf).
Primary lymphedema can be isolated or syndromic (whose manifestations are more complex with a group of symptoms) and mainly affects the lower limbs, or, much more rarely, upper limbs or external genitalia. Women are more frequently affected than men, preferentially young. The diagnosis is clinical, associating mild or non-pitting edema and skin thickening, as confirmed by the Stemmer’s sign (impossibility to pinch the skin on the dorsal side or the base of the second toe), which is pathognomonic of lymphedema. Limb lymphoscintigraphy is useful to confirm the diagnosis. Other causes of swelling or edema of the lower limbs must be ruled out, such as lipedema. The main acute lymphedema complication is cellulitis (erysipelas). Functional and psychological repercussions can be major,
deteriorating the patient’s quality of life. Treatment aims to prevent those complications, reduce the volume with low-stretch bandages, then stabilize it over the long term by exercises and wearing a compression garment. Patient education (or parents of a child) is essential to improve observance.
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Despite the high prevalence of allergic diseases in childhood, asthmatic children's parents/caregivers, elementary school teachers and university students have inadequate levels of knowledge to monitor these patients.
Background
Primary lower limb lymphedema is a chronic debilitating disorder without curative treatment. The initial treatment phase is dedicated to reducing lymphedema volume, whereas the second aims to stabilize that volume.
Objective
The objective of this study was to analyze clinical and lymphoscintigraphic characteristics during complete decongestive physical therapy as predictors of primary unilateral lower limb lymphedema-volume reduction.
Design
This observational, retrospective study included 222 consecutive patients (January 2009–January 2017; median age: 45.8 years) with lymphedema affecting the entire lower limb, who received complete decongestive physical therapy for the first time in a specialized lymphedema management center.
Methods
Complete decongestive physical therapy consisted of low-stretch bandaging, manual lymph drainage, exercises, and skin care for all patients. Lymphoscintigraphy preceded treatment.
Results
Median lymphedema evolution was 73 months, and median excess volume was 34%. Median (interquartile range) lymphedema volumes were 2845 (1038–3487) mL before and 1276 (601–2195) mL after a median of 11 days of complete decongestive physical therapy, with 34% median reduction. Multivariate analyses retained age, body mass index >40 kg/m2, and previous cellulitis, as independently associated with lymphedema volume reduction. For each additional year of age, volume reduction increased 0.16%. Unexpectedly, log-transformed initial lymphedema volumes indicated a negative impact, that is, 4.95%, for each log-unit gain. Patients with previous cellulitis episode(s) obtained 6.9% and those with BMI >40 kg/m2 17.1% higher lymphedema volume reductions. Lower limb lymphoscintigraphy was available for 150 (67.6%) patients. Having dermal back flow was associated with greater lymphedema volume reduction than not (respectively, 39% vs 31%).
Limitations
This study was retrospective, and only 67.6% of patients underwent lymphoscintigraphy.
Conclusion
Our analysis identified clinical and scintigraphic predictors of primary lymphedema volume reduction for patients with unilateral disease. Lymphoscintigraphy helps confirm lymphedema and predict volume reduction. Further study is required to confirm these observations.
Objective: To determine the prevalence of allergic rhinitis and associated factors in adolescents and in their parents/guardians. Methods: A cross-sectional study, applying a standardized and validated written questionnaire. Adolescents (13–14 years old; n=1,058) and their parents/guardians (mean age=42.1 years old; n=896) living in the city of Uruguaiana, southern Brazil, responded to the Global Asthma Network standard questionnaires. Results: The prevalence of allergic rhinitis in adolescents was 28.0%, allergic rhinoconjunctivitis, 21.3%, and severe forms of allergic rhinitis, 7.8%. In the adults, the prevalence of allergic rhinitis was 31.7%. Some associated factors with allergic rhinitis in adolescents include low physical exercise (OR 2.16; 95%CI 1.15–4.05), having only one older sibling (OR 1.94; 95CI 1.01–3.72) and daily meat consumption (OR 7.43; 95% CI 1.53–36.11). In contrast, consuming sugar (OR 0.34; 95%CI 0.12–0.93) or olive oil (OR 0.33; 95%CI 0.13–0 .81) once or twice a week, and eating vegetables daily (OR 0.39; 95%CI 0.15–0.99) were considered factors negatively associated. In adults, exposure to fungi at home (OR 5.25; 95%CI 1.01–27.22) and consumption of meat once or twice a week (OR 46.45; 95CI 2.12–1020.71) were factors associated with the medical diagnosis of allergic rhinitis, while low education (OR 0.25; 95%CI 0.07–0.92) was found to be a factor negatively associated. Conclusions: The prevalence of allergic rhinitis in adolescents is high, as well as its medical diagnosis in adults living in Uruguaiana. Environmental factors, especially food habits, were associated with findings in both groups.
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