Primary intestinal lymphangiectasia in an adult patient: A case report and review of literature.

BACKGROUND: Primary intestinal lymphangiectasia (PIL), first described in 1961, is a rare disorder of unknown etiology resulting in protein-losing enteropathy. The disease is characterized by dilatation and leakage of intestinal lymph vessels leading to hypoalbuminemia, hypogammaglobulinemia, and lymphopenia. Since the severity and location of lymph vessels being affected can vary considerably, the range of associated symptoms is wide from mild lower-limb edema to generalized edema, abdominal and/or pleural effusion, and recurrent diarrhea, among others. Although usually developing in early childhood, we present the case of a 34-year-old woman with PIL. Moreover, we performed a literature review systematically assessing clinical presentation, and provide a practical approach to facilitate diagnosis and therapy of PIL in adults. CASE SUMMARY: Our patient presented with unspecific symptoms of abdominal discomfort, fatigue, nausea, and recurrent edema of the lower limbs. Interestingly, a striking collinearity of clinical symptoms with female hormone status was evident. Additionally, polyglobulia, hypoalbuminemia, hypogammaglobulinemia, and transient lymphocytopenia were evident. Due to suspicion of a bone marrow disease, an extensive diagnostic investigation was carried out excluding secondary causes of polyglobulinemia and hypoalbuminemia. The diagnosis of primary intestinal lymphangiectasia was established after 22 wk by histological analysis of biopsy samples obtained via enteroscopy. Consecutively, the patient was put on a high-protein and low-fat diet with medium-chain triglycerides supplementation leading to significant improvement of clinical symptoms until 2 years of follow-up. CONCLUSION: PIL can be the reason for cryptogenic hypoalbuminemia, hypogammaglobulinemia, and lymphopenia in adulthood. Due to difficulty in correct diagnosis, treatment initiation is often delayed despite being effective and well-tolerated. This leads to a significant disease burden in affected patients. PIL is increasingly been recognized in adults since the majority of case reports were published within the last 10 years, pointing towards an underestimation of the true prevalence. The association with female hormone status warrants further investigation.

Investigating the Voce Faringea: Physiological and Acoustic Characteristics of the Bel Canto Tenor's Forgotten Singing Practice.

Several historical sources from the first half of the 19th century mention a distinct third register mechanism particular to tenor voices of that period. This so-called voce faringea-often described as an "intermediate" register-is a virtually forgotten historical singing practice used to extend the upper range of the voice, where the singer modifies falsetto, typically a weak and often feminine sound, into a more powerful, tenor-like vocal quality. Based on an evaluation of historical voice register theories, training strategies, and the sound ideals of the historical period, an informed discussion of that technique is developed. For this study, acoustic and electroglottographic signals for tones produced on the vowel /a/ by a professional tenor/countertenor in different vocal register mechanisms-voce faringea, falsetto, chest register, and mezza voce-were recorded using the VoceVista system. Analysis of the electroglottography (EGG) and audio data revealed specific characteristics of the voce faringea with regard to both the laryngeal mechanism and the sound spectrum, including high EGG contact quotient and low speed quotient values. EGG pulses were skewed significantly to the left and displayed a distinct knee shape during the de-contacting phase of the vocal folds, which consequently indicates a vibration with a clear mucosal wave. The long-term average spectrum and power spectrum exposed a considerable amplification and dislocation of F2 in the direction of high frequencies, thus boosting the third harmonic and showing a strong concentration of acoustic energy in the area of the singer's formant cluster.