Ultraviolet A phototest positivity is associated with higher free erythrocyte protoporphyrin IX concentration and lower transferrin saturation values in erythropoietic protoporphyria.

BACKGROUND: Erythropoietic protoporphyria (EPP) is a rare disorder of heme biosynthesis hallmarked by early-onset photosensitivity and mainly due to defective ferrochelatase activity leading to increased erythrocyte protoporphyrin IX (PPIX) levels. Evidence regarding the relationship between erythrocyte PPIX concentration and photosensitivity is limited. METHODS: To investigate the relationship between free erythrocyte PPIX (FEP) concentration; routine laboratory tests, particularly iron metabolism biomarkers; and ultraviolet (UV) A/visible light phototesting findings, 20 genetically confirmed EPP and one XLPP treatment-naive patients were included in our study. They underwent UVA and visible light phototesting. On the same day, blood samples were collected for measurement of FEP, serum iron, transferrin, transferrin saturation, and ferritin, 25-hydroxyvitamin D, and liver enzyme levels. RESULTS: Median FEP concentration at the time of phototesting was 57.50 (IQR: 34.58-102.70) µg/g of Hb. UVA and visible light phototesting were positive in 9 (42.9%) and 8 (38.1%) patients, respectively. Median FEP concentration was significantly higher in UVA phototest-positive patients than in those negative (64.37 [IQR: 57.45-121.82] vs 45.35 [IQR: 24.53-74.61] µg/g of Hb, respectively; P = .04486). Similarly, UVA photosensitive individuals had significantly lower median serum iron levels (61.5 [IQR: 33.5-84] µg/dL vs 109 [IQR: 63.25-154] µg/dL, respectively; P = .01862) and transferrin saturation values (15.005 [IQR: 7.0775-18.41] % vs 29.645 [IQR: 17.8225-34.3575] %; P = .0109) than those negative. CONCLUSIONS: Our study demonstrates that UVA phototest positivity is associated with higher FEP concentration and lower transferrin saturation and serum iron concentration in EPP.

Single-Centre Real-Life Experience of Guselkumab in Patients with Moderate-to-Severe Plaque Psoriasis.

The efficacy of guselkumab has been demonstrated in randomized controlled trials, but real-life data are still missing. This study presents a single-center real-life experience in treating moderate-to-severe plaque psoriasis with guselkumab, underlying the efficacy and safety of this biological agent. Primary efficacy endpoint was the percentage of patients reaching psoriasis area and severity index (PASI) 90 response at week 28. The cohort included 79 patients of mean age 51 ± 15 years. Mean duration of guselkumab treatment was 16,5 ± 7.5 months. All 79 patients completed at least 28 weeks of treatment. At week 28, 73 (92%) patients reached PASI 50 or higher, 65 (82%) achieved PASI 75 or higher, 54 (68%) achieved PASI 90 or higher, and 35 (44%) reached PASI 100. Very similar percentages were found at week 44 and at the end of the study period. No significant adverse effects were reported. This study confirms that guselkumab is an effective and safe biologic agent capable of maintaining long-lasting clinical response efficacy in real-world clinical practice, although at slightly lower levels than in clinical trials. J Drugs Dermatol. 2022;21(8):864-866. doi:10.36849/JDD.6962.

Adenoid cystic carcinoma of trachea: long-term disease control after endoscopic surgery and radiotherapy.

Aim: Adenoid cystic carcinoma is a rare tumor of head and neck region and its development in the thoracic region is even less frequent. This implies the absence of guidelines for therapeutic management and a consequent case-by-case approach. The role of radiotherapy is not yet clearly defined, but intensity-modulated radiotherapy allows for improved organ-at-risk sparing. Materials & methods: We have collected the cases of four patients treated at our institutions by the means of intensity-modulated radiotherapy, after endoscopic resection. Results & conclusion: Patients treated achieved long-term disease control of about 5 years, with a minimal acute toxicity. Longer follow-up is needed to drain conclusion on the impact of this treatment on overall survival.

Single-institution experience of intensity-modulated radiotherapy for malignant pleural mesothelioma at University of Catania.

AIM: The multimodal approach to malignant pleural mesothelioma is gradually becoming the standard of care for this disease in patients with good performance status. Materials & methods: We report our experience concerning eight cases treated with the use of static step-and-shoot intensity-modulated radiotherapy to the whole pleural cavity, in patients already undergoing surgical and/or antiblastic therapy. Results & conclusion: Results at a median follow-up of 16 months showed a median survival from the initial treatment of 29 months, with lung toxicity of grade II reported only in two patients.

Giant major aphtha.

Major aphthae are usually located on the dorsum of the tongue, the mucosal surface of the lips and the palate. They are large, round or oval ulcers, with a whitish-grey bed, well-defined borders and erythematous halo. They are very often accompanied by severe pain. Major aphthae can take up to four months to heal, often with a scar. Relapses are possible. We present a case of giant major aphtha that was previously diagnosed as squamous cell carcinoma.

Clinical Outcomes of Hydrogel Spacer Injection Space OAR in Men Submitted to Hypofractionated Radiotherapy for Prostate Cancer.

BACKGROUND/AIM: To evaluate the clinical outcomes of men with prostate cancer (PCa) submitted to hydrogel spacer injection before hypofractionated radiotherapy (HRT). PATIENTS AND METHODS: From April 2018 to April 2020, 32 patients with clinically localized PCa underwent hydrogel injection Space OAR before HRT to the prostate and seminal vesicle; the prescription dose was 60 Gy in 20 fractions, 5 days/week over 4 weeks. PSA levels, genitourinary (GU) and gastrointestinal (GI) toxicities, and sexual function were prospectively evaluated. RESULTS: PSA levels at the median follow up of 15 months was 0.52 ng/ml; 28.1% vs. 78.1% patients had GI vs. GU Grade 0 acute toxicity and 93.7% vs. 0% had GI vs. GU Grade 0 late toxicity. Furthermore, 88.1% of patients kept pretreatment sexual potency. CONCLUSION: The use of the hydrogel Spacer OAR before HRT is useful for reducing acute and late GU and GI toxicities.

Breast cancer bone metastases: an epidemiologic study in selected radiation departments.

AIMS: To quantify radiation treatments for bone metastases from breast cancer in three Sicilian provinces (Messina, Catania, Ragusa) with respect to the general population and the incidence and prevalence of breast cancer in this macro-area in a 8-year period. METHODS: All bone metastasis patients treated using radiotherapy in 8 radiation departments operating in the provinces of Messina, Catania and Ragusa were collected. Among these, metastases from breast carcinoma were analyzed according to year of irradiation. An analysis of breast cancer incidence and prevalence with respect to inhabitants in this macro-area was conducted using literature and census data. RESULTS: From January 2004 to December 2011, irradiation was delivered in 4419 bone metastasis patients. Among these, 1617 had a primary breast cancer with a median treatment per year of 206.5 (range, 164-251); 211 patients were treated in 2004 and 206 in 2011. In 2004, there were 1,048,957 female residents in the three provinces and 1,065,422 in 2011. The reported breast cancer incidence and prevalence in Sicily was respectively 100.79/100,000 and 908.54/100,000 in 2004 and 108.41/100,000 and 1091.29/100,000 in 2010. CONCLUSIONS: There has been an increase in both incidence and prevalence of breast cancer in Sicily. Nevertheless, it seems that there was no increment in skeletal-related events requiring irradiation in such patients in eastern Sicily radiation departments.