NORMALIZING EFFECT OF GAMMA-LINOLENIC ACID ON ETRETINATE-INDUCED HYPERTRIGLY- CERIDEMIA IN PSORIATIC PATIENTS: PRELIMINARY RESULTS

_________________ Synopsis Retinoids are a group of. synthesis compounds having vitamin A as their precursor. Among retino i d s, etretin ate i s commonly used, al o n e or in combi n ati o n with PUVA (8-methoxypsoralen+UYA), for diffuse and resistant psoriasis. However, eretrinate may induce locai (severe ski n dryness, flaking, cheilitis and fiss ures) and systemic side-effects. Among the systemic side-effects of etretinate, increased triglycerides, VLDLs, and cholesterol are freq ue ntly detected, whic h may cause treatment discontinuation. In the present paper 41 psoriatic patients undergoing Re-PUVA (Etretinate+PUVA) were considered. One month after the beginning of the therapy, 27 of them were additionally treated with gamma-linolenic acid, because of the dryness and itch induced by etretinate. During Re-PUVA treatment 11 out of these 27 patients showed increased triglyceride and cholesterol levels. A 2 month-treatment with gamma-linolenic acid induced an improvement of the cutaneous side-effects of eretrinate. Moreover, a statisticall y significant decrease of triglyceridemia (p<O.O I) and cholestero lemia (p<O.O I ) was detected followi ng the treatment with gamma-linolenic acid. These results demonstrate that association of gamma-linolenic acid is useful in contolling dyslipidemia if Re-PUVA treatment has to be continued to achieve clearing of psoriasis. ------------------Riassunto I retinoidi sono una famiglia di composti di sintesi che hanno il loro precursore nella Yit. A. I più diffusi in ambito terapeutico dermatologico sono l'etretinato, l'isotretinoina e l'acitretina. Questi farmaci agiscono promuovendo la differenziazione cheratinocitaria ed esercitano un effetto immunomodulatore. In particolare, l'etretinato trova indicazione, da solo o in associazione con PUVA-terapia (8-metossipsoralene+UYA), nel trattamento delle forme più estese e resistenti di psoriasi. L'etretinato, tuttavia, può indutTe effetti collaterali sia locali (secchezza cutanea marcata, desquamazione, cheilite e ragadi) che generali. Tra gl i effetti sistemici collaterali c he l'etretrinato può indun-e si ri leva più frequentemente un innalzamento dei trigliceridi, delle VLDL e del colesterolo che possono indun-e a sospendere la terapia prima del conseguimento di una risposta clinica soddisfacente. Nel presente lavoro vengono riportati i dati relativi al contollo dell' iperlipemia indotta da etretinato me- 107 Norma/1zmg effect of gamma-linolenic acid on etretinate-finduced hypertriglyceridemia in. .. diante l'impiego di acido gamma-linolenico per via generale. Tale acido grasso essenziale è stato impiegato in epoca recente per il controllo del prurito e della secchezza cutanea nei soggetti affetti da dermatite atopica; per questo motivo abbiamo ritenuto di utilizzarl o (480 mg/os/die) in un gruppo di 27 pazienti ps oriasici che, in cor so di tra ttame nto co n Etretinato (Etretinato 0,5 mg/Kg/os/die)+PUVA (Re-PUVA), avevano manifestato marcata secchezza cutanea e prurito, imputabili al retinoide. Ad un mese dall'inizio della terapia, 11 dei 27 pazienti affetti da secchezza cutanea e prurito avevano presentato anche anomalie del quadro lipidico, imputabili al trattamento con etretinato. II trattamento con acido gamma-linolenico per 2 mesi, o ltre a determ inare un miglioramento degli effetti collaterali cutanei, ha indotto anche una ridu zione statisticamente significativa della trigl iceridemia (p<0,01) e della colesterolemi a (p<0,0 1). Pertanto anche se gli innalzamenti di colesterolo e trigliceridi nella nostra casistica sono risultati, durante al terapia con etretinato, di moderata entità, l'util izzo d i acido gamma-linolenico ci ha permesso di continuare la terapia Re-PUVA con una certa sicurezza per il paziente. Come noto, infa tti , la popolazione psoriasica risu lta esposta ad un aumentato rischio di accidenti cardiovascolari, in associazione a diabete ed obesità, e l'ipertrig liceridemia iatrogena costituirebbe quindi un ulteriore fattore di ri schio

Long-term disease-free survival in surgically-resected oral tongue cancer: a 10-year retrospective study

Sopravvivenza libera da malattia a lungo termine nel tumore della lingua mobile operato: studio retrospettivo a 10 anni.PLAIN-LANGUAGE-SUMMARY: Early and loco-regionally advanced oral tongue squamous cell carcinoma (OTSCC) can be treated by surgery alone or followed by adjuvant radiotherapy or chemoradiotherapy. Nevertheless, up to 40% of patients develop tumour relapse. The aim of our study is to investigate the clinical and pathological features associated with reduced disease-free survival (DFS) in a cohort of surgically-resected OTSCC patients. One hundred and six patients surgically resected for OTSCC were retrospectively identified from clinical records. DFS was calculated according to the Kaplan\u2013Meier method and differences between variables were assessed with Log-Rank test. A multivariable Cox regression model was used to analyse the impact of different prognostic factors on DFS. After a median of follow-up of 8.9 years, 22 events, including 11 deaths, were observed. Overall, the 5-year DFS-rate was 87.4%. The presence of extra-nodal extension (p = 0.023) and perineural invasion (p = 0.003) were significantly correlated with shorter DFS (in univariate analysis). In multivariable analysis, extra-nodal extension and perineural invasion confirmed their role as independent prognostic factors associated with an increased risk of disease recurrence [hazard ratio (HR) 2.87, 95% CI 1.11-7.42, p = 0.03; HR 3.85, 95% CI 1.49-9.96, p = 0.006, respectively]. p16 and p53 expressions in tumour cells were detected in 12% (n = 9) and 46% (n = 40) of cases, respectively. No differences in DFS were observed between p16+ and p16- (p = 0.125) and between p53+ and p53- tumours (p = 0.213). In conclusion, radical surgery, eventually followed by adjuvant radiotherapy or chemo-radiotherapy, can achieve high cure rates in OTSCC. After long-term follow-up, perineural invasion and extra-nodal extension confirmed their role as prognostic factors associated with reduced DFS in OTSCC patients

Technical refinements in mandibular reconstruction with free fibula flaps: Outcome-oriented retrospective review of 99 cases = Accorgimenti tecnici nelle ricostruzioni mandibolari con lembi liberi di fibula: analisi retrospettiva dei risultati su 99 casi

Congenital disease, major trauma, tumour resection and biphosphonate-related osteonecrosis can lead to partial, subtotal, or total loss of the mandibular bone. Minor defects can be easily reconstructed using bone grafts, whereas microvascular free tissue transfer may be unavoidable in the case of major bone loss or poor quality of soft tissue. Simple bone or composite osteocutaneous fibula free flaps have proven invaluable and remain the workhorse for microvascular mandibular reconstruction in daily practice. Our experience with 99 consecutive fibular free flaps confirms the available data in terms of high success rate. In these cases, 90% had total success, while 7 had complete flap failures. Three of our patients showed skin paddle necrosis with bony conservation. This report focuses on the technical refinements used by the authors that can prove valuable in obtaining predictable and precise results: in particular, we discuss surgical techniques that avoid vascular pedicle ossification by removing the fibular periosteum from the vascular pedicle itself and reduce donor site morbidity and aid in management of the position in the new condylar fossa. Finally, new technologies such as intraoperative CT and custom premodelled fixation plates may also increase the predictability of morpho-functional results

Congenital hypothyroidism with eutopic thyroid gland : analysis of clinical and biochemical features at diagnosis and after re-evaluation

Context: In recent years changes in screening strategies for congenital hypothyroidism (CH) led to an increased detection of mild forms of CH, associated with eutopic thyroid gland. Objectives: We aimed to determine the clinical evolution of CH with eutopic thyroid gland and to find out prognostic factors at diagnosis and follow-up. Patients and Methods: We retrospectively analyzed agroup of84 children withCH andeutopic thyroid gland treated at our institution. They all underwent clinical re-evaluation after the age of 3, based on thyroid function testing after L-thyroxine therapy withdrawal, thyroid ultrasonography, and 123I scintigraphy with perchlorate discharge test. Genetic analysis was performed in selected cases. Results: At re-evaluation, 34.5% of patients showed permanent hypothyroidism and needed L-thyroxine reintroduction, 27.4% had persistent hyperthyrotropinemia (TSH5-10mU/L), and 38.1% had transient hypothyroidism. Major risk factors for permanent CH were prematurity, first-degree familial history of goiter/nodules, thyroid hypoplasia at diagnosis, and high L-thyroxine requirements at follow-up. Iodine organification defects were found in 29.7% of patients, 30% of whom harbored DUOX2 mutations. TSH receptor gene mutations were found in 8.7% of patients with persistent thyroid dysfunction and negative perchlorate discharge test. Conclusions: Only one-third of patients with CH and eutopic thyroid gland needed to continue L-thyroxine therapy after re-evaluation. A frequent finding was the persistence of mild hyperthy-rotropinemia. The evolution of CH remains difficult to predict, although different clinical features might suggest different outcomes. Mutations in the genes commonly linked to mild forms of CH were documented in a minority of cases. Copyrigh

Werner's syndrome: seven cases in one family.

7 cases of Werner's syndrome in one family of northern Sardinia (the female : male ratio being 4:3) are reported. A 9-year-old girl affected with Cooley's anemia is reported too. The typical complete pattern of the syndrome was observed in patients in the fourth decade of their lives, whereas in the two youngest ones, some features were missing. 1 patient died of gastric carcinoma, 1 of cachexia. Consanguinity was established in two generations. The genealogical tree suggests an autosomal recessive mode of inheritance. Genealogical, clinical, biochemical, and histopathological studies were performed. As far as we know, this is the largest number of patients with Werner's syndrome reported in one family

Familial multiple lipomatosis

[No abstract available

Familial multiple lipomatosis.

A clinical study was made of 14 cases of multiple symmetrical lipomata in two families. There were 7 female and 7 male patients. In one family the members affected were observed in four generations. The disease set in during the third or fourth decade of life. The lipomata ranged in size from that of a pea to that of a hen's egg. They were limited to the forearms and trunk were asymptomatic