RESUMEN
PURPOSE: To describe the urological manifestations of Erdheim-Chester disease (ECD) and their computed tomography (CT) findings. METHODS: We retrospectively reviewed 48 patients diagnosed with ECD at Peking Union Medical College Hospital from January 2014 to January 2020. Twenty-four patients exhibited urological manifestations. Their CT findings, including appearances of the involved area (e.g., perirenal space, renal sinus, ureters, renal arteries, and adrenal glands), occurrence rate of ECD involvement in each area, signal enhancement pattern after CT contrast agent administration, disease progression, and causes of hydronephrosis were discussed. RESULTS: In 24 patients with evidence of ECD urological involvement, the most common manifestation was perirenal infiltration, appearing as "hairy kidney" on unenhanced CT scans and moderate signal enhancement on enhanced CT scans (17/24, 70.8%). Other manifestations included renal sinus infiltration (16/24, 66.7%), proximal ureter involvement (14, 58.3%), renal artery sheath (10, 41.7%), hydronephrosis (14, 58.3%), and adrenal glands involvement (8, 33.3%). The histiocytic infiltrate was mostly bilateral, starting from the perirenal space and spreading to the renal sinus and ureters. Hydronephrosis was usually associated with infiltration of ureters. CONCLUSION: Kidneys are the most common visceral organs affected by ECD. CT scanning is not only advantageous in early diagnosis, but also critical for designing the treatment regime for patients with ECD.
Asunto(s)
Enfermedad de Erdheim-Chester , Hidronefrosis , Enfermedad de Erdheim-Chester/complicaciones , Enfermedad de Erdheim-Chester/diagnóstico por imagen , Humanos , Cintigrafía , Estudios Retrospectivos , Tomografía Computarizada por Rayos XRESUMEN
Diffuse panbronchiolitis (DPB) is a chronic diffuse airway inflammatory disease, which is strongly associated with the class I human leukocyte antigen (HLA) alleles. Here, we report a pair of sisters who have been suffering from chronic cough, expectoration and wheezing for many years. They were previously misdiagnosed as chronic bronchitis and bronchial asthma, and were recently diagnosed as diffuse panbronchiolitis. The 36-year-old elder sister suffered from diffuse panbronchiolitis complicated with pulmonary tuberculosis. The 30-year-old younger sister suffered from diffuse panbronchiolitis complicated with bronchial asthma and bronchiectasis. We have performed HLA genotyping research on the two sisters, their parents and younger brother. The results showed that all family members were positive for HLA-A24 and HLA-B13. The younger sister and mother were positive for HLA-A2. The younger brother and father were positive for HLA-A11. We suspect that the two sisters' disease susceptibility may be caused by their parents' consanguineous marriage. In this study, we reported the clinical characteristics of the two sisters with diffuse panbronchiolitis and shared the associated HLA genotyping results of this family cluster, hoping to provide reference for the etiology, diagnosis and treatment of this disease.
Asunto(s)
Bronquiolitis , Predisposición Genética a la Enfermedad , Adulto , Anciano , Bronquiolitis/diagnóstico , Bronquiolitis/genética , China , Consanguinidad , Infecciones por Haemophilus , Humanos , MasculinoRESUMEN
Objective To investigate the computed tomography(CT)imaging features of IgG4-related kidney disease(IgG4-RKD).Methods The clinicopathological and imaging data of 36 IgG4-RKD patients(including 26 cases of renal parenchyma,10 cases of renal pelvis,24 cases of double kidney or multiple lesions,and 12 cases of single focus)were retrospectively analyzed.Results IgG4-RKD had specific clinicopathological and imaging features.Although the kidney or renal pelvis was involved,there were no common clinical manifestations of malignant tumors such as hydronephrosis and hematuria.The boundary was clear and complete,and it had typical characteristics of continuous progressive enhancement.The peak value was mostly in the solid and excretory phase,and there were no imaging manifestations such as liquefaction,necrosis,cystic degeneration,and calcification.Even if the renal pelvis was obviously involved and the focus was large,there was no invasion of the peripheral renal parenchyma,the shape of the renal pelvis still existed,the wall was smooth,and there was no enlarged peripheral lymph nodes.Conclusions CT images of IgG4-RKD are similar to those of renal malignant tumors.Based on clinicopathological features,CT findings,and blood IgG4-related tests,a definite diagnosis can be made and unnecessary operation can be avoided.