The efficacy and safety of intra-articular injection of triamcinolone acetonide versus triamcinolone hexacetonide for treatment of juvenile idiopathic arthritis.

OBJECTIVES: Juvenile idiopathic arthritis (JIA) is the most common childhood rheumatic disease. Intra-articular corticosteroids joint injection (IAJI), with triamcinolone hexacetonide (TH) or triamcinolone acetonide (TA), is an effective additional treatment for oligo and polyarticular JIA. Previous studies have shown the benefits of TH over TA; however, TA is still used in many pediatric rheumatology centers. Our unit has experience with both regimens, and therefore we aimed to compare the efficacy and safety of TA versus TH for JIA patients. METHODS: Chart review of JIA patients who were randomly (based on drug availability) treated with TA or TH IAJI during 2010-2019. Primary outcomes for efficacy were defined as full recovery from arthritis one month after IAJI and a relapse rate of arthritis 3 months after IAJI. Primary outcome for safety was defined as the occurrence of adverse events (AEs) during the follow up period after IAJI. RESULTS: Overall, 292 joints of 102 JIA patients were treated (138 TA/154 TH joints). Complete recovery after one month was documented in 107 (69.6%) of TA treated joints and 96 (69.5%) of TH treated joints (P = 0.232). However, rate of relapse after 3 months was significantly higher for TA treated joints (27 (20.1%) vs. 13 (8.8%), respectively, P < 0.01). No AEs were documented except minor scars at four joint injection sites. CONCLUSION: The recovery from arthritis was similar (~ 70%) with both regimens, however relapse rate was more than double in TA as compared to TH injected joints. These findings are important due to a contemporary shortage of TH in the US market.

The effect of narrow-band ultraviolet B radiation on sleep, happiness, and appetite: A prospective cohort study.

BACKGROUND: The health benefits of sunlight are well documented, including the effects on happiness and sleep. However, only a few studies have investigated the benefits of artificial narrow-band ultraviolet B (NB-UVB) radiation. OBJECTIVES: To investigate the effect of NB-UVB on sleep quality, happiness, and appetite. METHODS: Patients from a single phototherapy unit were selected, and their epidemiological characteristics were documented. Subjects were asked to complete questionnaires including the Pittsburg Sleep Quality Index (PSQI), Subjective Happiness Scale (SHS), and the Simplified Nutritional Appetite Questionnaire (SNAQ) 4 weeks before and after the initiation of the NB-UVB therapy. The sample consisted of 52 patients. RESULTS: Four weeks after the initiation of NB-UVB sessions, sleep quality was significantly improved (the PSQI decreased from 6.5 to 5.23 (t = -3.52, P < .01). In contrast, subjective happiness did not improve (the SHS decreased from baseline 5.5 to 5.2, P > .05). Similarly, appetite did not change (the SNAQ score increased from baseline 14.75 to 15.05, P > .05) and body mass index was not affected by phototherapy. In general, men were found to have better sleep quality before and after UV light therapy. CONCLUSION: Our data suggest that NB-UVB exposure over 4 weeks improves sleep quality; however, it does not influence happiness or appetite.

Familial Periodic Fever, Aphthous Stomatitis, Pharyngitis and Adenitis Syndrome; Is It a Separate Disease?

Introduction: Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA) is the most common periodic fever syndrome in the pediatric population, yet its pathogenesis is unknown. PFAPA was believed to be sporadic but family clustering has been widely observed. Objective: To identify demographic and clinical differences between patients with PFAPA and a positive family history (FH+) as compared to those with no family history (FH-). Methods: In a database comprising demographic and clinical data of 273 pediatric PFAPA patients treated at two tertiary centers in Israel, 31 (14.3%) had FH+. Data from patients with FH+ were compared to data from those with FH-. Furthermore, family members (FMs) of those with FH+ were contacted via telephone for more demographic and clinical details. Results: The FH+ group as compared to the FH- group had more myalgia (56 vs. 19%, respectively, p = 0.001), headaches (32 vs. 2%, respectively, p = 0.016), and a higher carrier frequency of M694V mutation (54% vs. 25%, respectively, p = 0.05). Colchicine was seen to be a more beneficial treatment for the FH+ group as compared to the FH- group; however, with no statistical significance (p = 0.096). FMs displayed almost identical characteristics to patients in the FH+ group except for greater arthralgia during flares (64 vs. 23%, respectively, p = 0.008), and compared to the FH- group they had more oral aphthae (68 vs. 43%, respectively, p = 0.002), myalgia/arthralgia (64 vs. 19%/16%, respectively, p < 0.0001), and higher rates of FH of Familial Mediterranean fever (FMF) (45 vs.15%, respectively, p = 0.003). Conclusions: Our findings suggest that patients with a FH+ likely experience a different subset of disease with higher frequency of family history of FMF, arthralgia, myalgia, and might have a better response to colchicine compared to FH-. Colchicine prophylaxis for PFAPA should be considered in FH+.

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome - Is It Related to Ethnicity? An Israeli Multicenter Cohort Study.

OBJECTIVES: To evaluate the ethnic distribution of Israeli patients with the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA). STUDY DESIGN: The medical records of patients with PFAPA attending 2 pediatric tertiary medical centers in Israel from March 2014 to March 2019 were retrospectively reviewed. Patients with concomitant familial Mediterranean fever were excluded. Ethnicity was categorized as Mediterranean, non-Mediterranean, and multiethnic. Findings were compared with patients with asthma under treatment at the same medical centers during the same period. RESULTS: The cohort included 303 patients with PFAPA and 475 with asthma. Among the patients with PFAPA, 178 (58.7%) were of Mediterranean descent (Sephardic Jews or Israeli Arabs), 96 (33.0%) were multiethnic, and 17 (5.8%) were of non-Mediterranean descent (all Ashkenazi Jews). Patients with PFAPA had a significantly higher likelihood of being of Mediterranean descent than the patients with asthma (58.7% vs 35.8%; P < .0001). The Mediterranean PFAPA subgroup had a significantly earlier disease onset than the non-Mediterranean subgroup (2.75 ± 1.7 vs 3.78 ± 1.9 years, P < .04) and were younger at disease diagnosis (4.77 ± 2.3 vs 6.27 ± 2.9 years, P < .04). CONCLUSIONS: PFAPA was significantly more common in patients of Mediterranean than non-Mediterranean descent. Further studies are needed to determine the genetic background of these findings.

Rhabdomyosarcoma disease spread evaluation on CT scans: Association with primary tumor size and Ki-67 proliferation marker.

PURPOSE: Ki-67 is a marker of cellular proliferation that is commonly used for the assessment of rhabdomyosarcoma. The aim of this study was to investigate the associations between Ki-67 expression and primary tumor diameter with CT evidence of lymph node and solid organ metastatic spread in rhabdomyosarcoma. MATERIALS AND METHODS: An institutional review board approval was granted for this study. A retrospective search for rhabdomyosarcoma patients was conducted. Pathology reports were examined for Ki-67 expression. Chest-abdomen CT was assessed for radiological evidence of lymph node and metastatic spread. The maximal primary tumor diameter (termed tumor size) was also measured in different modalities CT, MRI, PET-CT and US. Ki-67 levels and primary tumor maximal diameters were compared to CT evidence of lymph node and organ metastatic spread. RESULTS: Twenty-four patients with rhabdomyosarcoma were included. CT evidence of lymph node spread was associated with Ki-67 levels (AUC = 0.896, p = 0.006) and to a lesser extent with tumor size (AUC = 0.790, p = 0.030). However, organ metastatic spread was associated only with tumor size (AUC = 0.854, p = 0.006) and not with Ki-67 levels (AUC = 0.604, p = 0.469). A combination of tumor size ≥50 mm and Ki-67 levels ≥60% was significantly associated with CT evidence of lymph node spread (p = 0.004). CONCLUSION: In conclusion, this study demonstrates radiological-pathological correlation in RMS. Lymph node spread detected by radiological images is associated with Ki-67 values. Lymph node and metastatic spread are associated with primary tumor size.

Overuse of CT and MRI in paediatric emergency departments.

The aim of this review is to survey CT and MRI overuse in the paediatric emergency department (ED) population. CT is one of the most important modalities employed in the ED. Not surprisingly, its high accuracy, rapid acquisition and availability have resulted in overuse. An obvious limitation of CT is ionizing radiation; in addition there are economic implications to overuse. Studies from the last two decades have shown increase in paediatric ED CT utilization in the first decade, reaching a plateau forming around 2008, followed by a decrease in the last decade. This decrease occurred in conjunction with campaigns raising awareness to the risks of radiation exposure. Although a trend of decrease in overuse have been observed, great variability has been shown across different facilities, as well as among physicians, with more pronounced overuse in non-teaching and non-children dedicated EDs. The leading types of paediatric ED CTs are head and abdominal scans. Decision rules, such as PECARN for head injury and the Alvarado score for abdominal pain, as well as using alternative imaging modalities, have been shown to reduce CT overuse in these two categories. MRI has the obvious benefit of avoiding radiation exposure, but the disadvantages of higher costs, less availability and less tolerability in younger children. Although anecdotally paediatric ED MRI usage has increased in recent years, only scarce reports have been published. In our opinion, there is need to conduct up-to-date studies covering paediatric CT and MRI overuse trends, usage variability and adherence to clinical protocols.