ABSTRACT
BACKGROUND: Adult-onset Still's disease and systemic-onset juvenile idiopathic arthritis constitute two sides of the same continuum disease. We aimed to investigate the similarities and differences between those diseases. METHODS: We conducted a retrospective study including adult patients affected by still's disease, attending the rheumatology department and patients affected by systemic-onset juvenile idiopathic arthritis attending the pediatric department. We recorded clinical and radiological findings, different therapeutic regimens, and disease patterns. RESULTS: There were 8 adult patients (6 females and 2 males) and 8 juvenile patients (4 females and 4 males). The classical triad of spiking fever, arthritis, and evanescent skin rash was the first clinical presentation observed in 4 adult patients and in 2 juvenile patients. Arthritis was noted in 8 adult patients versus 6 juvenile patients. Joint deformities were seen in adult patients. Non-steroid antiinflammatory drugs and corticosteroids were the most prescribed molecules. csDMARDs and bDMARDs were used in second-line therapy only for adult patients. The monocyclic course was predominant in juvenile patients and the polycyclic course in adult patients. The chronic course was observed only in two adult patients. Remission was noted in 5 adult patients and 6 juvenile patients. There were no significant differences between the two groups regarding clinical findings, different therapeutic regimens, and disease patterns. CONCLUSION: From the findings of our study, it seems that AOSD and sJIA are the same syndrome continuum expressed in different hosts. This hypothesis is supported by clinical course, molecule evidence, cytokine profile, and treatment response.
Subject(s)
Arthritis, Juvenile , Still's Disease, Adult-Onset , Male , Child , Female , Humans , Adult , Arthritis, Juvenile/drug therapy , Retrospective Studies , Adrenal Cortex Hormones/therapeutic use , Still's Disease, Adult-Onset/drug therapy , Anti-Inflammatory Agents, Non-Steroidal/therapeutic useABSTRACT
AIM: To investigate risk factors of renal complications in glycogen storage disease type I, in order to identify practical implications for renal preservation. METHODS: A retrospective study of 38 patients with glycogen storage disease type I. RESULTS: The patients studied were 8.6 years old in average (1.5 to 22 years) and were followed during 7.4 ± 4.5 years. Hypercalciuria was detected in 23 patients and was related to acidosis (P=0.028), higher lactate levels (5.9 ± 3.5 versus 3.7 ± 1.7 mmol/L; P=0.013) and smaller height (-2.1 ± 1.5 SD versus -0.8 ± 1.5 SD; P=0.026). Urolithiasis was diagnosed in 7 cases. Glomerular disease (19/38) was more frequent in cases with severe hypertriglyceridemia (P=0.042) and occurred at an older age (P=0.007). Microalbuminuria occurred in 15/31 cases; ACE inhibitors were prescribed in only 8 cases. The frequency of renal complications did not differ according to the diet group (continuous enteral feeding or uncooked starch). Logistic regression concluded as risk factors: lactic acidosis for tubular disease and age>10 years for glomerular disease. CONCLUSIONS: Renal involvement is common in glycogen storage disease type I patients. Tubular abnormalities are precocious, related to lactic acidosis and may be detected by monitoring of urinary calcium. Glomerular hyperfiltration is the first stage of a progressive glomerular disease and is related to age. Practical implications for renal preservation are discussed based on our results and literature.
Subject(s)
Glycogen Storage Disease Type I/complications , Kidney Diseases/etiology , Acidosis, Lactic/complications , Adolescent , Adult , Age Factors , Child , Child, Preschool , Female , Follow-Up Studies , Humans , Infant , Male , Retrospective Studies , Risk Factors , Young AdultABSTRACT
Rosai-Dorfman disease (RDD) is a benign lymphoproliferative disorder characterized by cervical lymph node enlargement with a consistent risk of airway compression and esthetic damage. Extranodal localizations are also described. There is no therapeutic consensus for pediatric forms of RDD. Through 2 pediatric cases with nodal involvement in 1 patient and a sinonasal and soft tissue localization in the other, we focus on the management problems of both nodal and extranodal RDD.