scholarly journals Quality of life and clinical characteristics of self‐improving congenital ichthyosis within the disease spectrum of autosomal recessive congenital ichthyosis

Author(s):  
L. Hake ◽  
K. Süßmuth ◽  
K. Komlosi ◽  
J. Kopp ◽  
C. Drerup ◽  
...  
2019 ◽  
Vol 1 ◽  
pp. 101-103
Author(s):  
S. Anjana ◽  
Kunjumani Sobhanakumari ◽  
Rony Mathew ◽  
Rani Mathew

Collodion baby is a transient condition in newborns where they are covered in a taut, shiny membrane. Later, it can evolve into any form of autosomal recessive congenital ichthyosis or other ichthyosiform syndromes. Retinoids are one of the most effective therapeutic modalities for ichthyosis and have been found to be safe in neonates. Hence, early and judicious use of retinoids can significantly improve the quality of life in severe ichthyosis. Herein, we report a case of congenital ichthyosis which showed an excellent response to acitretin therapy.


2020 ◽  
Vol 48 (12) ◽  
pp. 030006052097987
Author(s):  
Umesh Jayarajah ◽  
Thisaru Widyarathne ◽  
Metthananda Nawarathne ◽  
Sivananthan Raguvaran ◽  
Nishanthan Subramaniam ◽  
...  

Objective We aimed to describe the clinical characteristics of chronic pancreatitis (CP) and patient quality of life (QOL) in a resource-limited setting. Methods We performed a cross-sectional study including patients with clinical and radiological features of CP. We collected clinical data and assessed QOL using the European Organization for the Research and Treatment of Cancer Quality of Life Questionnaire. Results We included 103 patients (median age 44 years, 84 men). Median age at symptom onset was 36 (4–78) years. Around 70% of patients had diabetes mellitus and 62.1% had consumed alcohol; 36 (35%) were current smokers. The mean overall global QOL score was 68.7. Most patients (91.3%) sought treatment from multiple centers. Nineteen (18.5%) had pancreatic stone disease, 38 (36.9%) had persistent abdominal pain (median severity 7.8/10, 59 (57.3%) had steatorrhea, and 56 (54.4%) had jaundice. Poor QOL was significantly associated with weight loss, loss of appetite, and intractable pain. No correlation with age, sex, or alcohol consumption was noted. Conclusion A considerable proportion of patients with CP had troublesome symptoms. Intractable pain, loss of appetite, and weight loss were significantly associated with poor QOL. Further assessment is needed of patients’ psychosocial well-being and its association with QOL.


2018 ◽  
Vol 8 (12) ◽  
pp. e01135
Author(s):  
Juan-Juan Du ◽  
Tian Wang ◽  
Pei Huang ◽  
Shishuang Cui ◽  
Chao Gao ◽  
...  

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