Itraconazole as 'bridge therapy' to anti-IgE in a patient with severe asthma with fungal sensitisation.

Sensitisation to fungi has been reported to play an important role in a particular phenotype of severe asthma, the so-called severe asthma with fungal sensitisation, characterised by high levels of total IgE, which may be an obstacle to anti-IgE therapy. We describe here the case of a polysensitised woman with refractory asthma, sensitised to Aspergillus fumigatus with high total IgE values (1793 kUA/l), but without the diagnostic criteria for allergic bronchopulmonary aspergillosis. Additional therapy with itraconazole leads to the decrease of total IgE to the limits recommended for proper omalizumab dosing (30–1500 kUA/l). Itraconazole, used as bridge therapy, provided us the opportunity to start anti-IgE treatment in a patient with high levels of total IgE, beyond the upper limits recommended for proper prescription of omalizumab

Xanthogranulomatous pyelonephritis: presentation and management

Xanthogranulomatous pyelonephritis (XGP) is characterized by the presence of lipid-laden foamy macrophages with both acute and chronic phase inflammatory cells. The aim of the study is to present our experience about patients with Xanthogranulomatous pyelonephritis. 29 patients were evaluated through a complete anamnesis and the preoperative management included routine blood and biochemical tests, urine culture and renal ultrasound, intravenous urography and computed tomography (CT). All patients underwent open nephrectomy followed by the pathological exam. The main symptoms of these patients were fever and flank pain. Preoperative laboratory tests revealed anemia, leukocytosis and increasing levels of blood urea nitrogen (BUN) and creatinine. Kidney failure was noticed in almost half of the cases. This study succeeded to evaluate the demographic, clinical, biological, surgical and histological characteristics. A pathological diagnosis is mandatory mainly for the evaluation of its coexistence with renal carcinoma

Tratamentul laparoscopic al herniei gastrice transhiatale – experienţa personală

Spitalul Clinic de Urgenţă “Bagdasar-Arseni” Bucureşti, România, Spitalul Clinic de urgenţă “Sf. Pantelimon” Bucureşti, România, Spitalul Judeţean de Urgenţă Piteşti, România, Universitatea de Medicină şi Farmacie “Carol Davila” Bucureşti, România, Al XI-lea Congres al Asociației Chirurgilor „Nicolae Anestiadi” din Republica Moldova și cea de-a XXXIII-a Reuniune a Chirurgilor din Moldova „Iacomi-Răzeșu” 27-30 septembrie 2011Obiective. Studiul de faţă işi propune să analizeze dificultăţile metodei laparoscopice în tratamentul chirurgical al herniei gastrice transhiatale, criteriile de includere pentru chirurgia laparoscopică, limitele metodei şi nu în ultimul rând acumularea experienţei în această privinţă prin curba de învăţare într-un serviciu de chirurgie generală ce a fost dotat cu linie de chirurgie laparoscopică. Material şi metodă. Studiul a fost realizat pe intervalul Iunie 2008 – Iunie 2011 pe un lot de 14 pacienţi, marea lor majoritate bărbaţi, la care s-a intervenit chirurgical laparoscopic pentru hernie gastrică transhiatală şi la care laparoscopia a reprezentat unica metodă de tratament. Am analizat pacienţii din punct de vedere al sexului, vârstei, ţărilor asociate şi am reuşit să stabilim un protocol intern de includere pentru chirurgia laparoscopică la nivelul spitalului nostru prin care am obţinut beneficiile maxime pentru pacient. Procedeele utilizate au fost intervenţiile Nissen si Dor. Rezultate. Din punct de vedere al rezultatului postoperator rezultatele sunt cel puţin comparabile cu cele din chirurgia clasică, dar laparoscopia are avantajul invazivităţii minime, duratei scurte de spitalizare, reintegrării socio-profesionale rapide şi, nu în ultimul rând, reducerea costurilor per ansamblu. Dificultăţile sunt de obicei întâlnite la momentul recalibrării hiatusului esofagian în cazul herniilor hiatale voluminoase când “defectul” este destul de larg. După o dotare corespunzatoare şi după parcurgerea curbei de învăţare, rezultatele postoperatorii sunt benefice, iar superioritatea metodei, mai ales în cazurile cu patologie asociată marcată, este evidentă. Concluzii. Caracterele expuse ale chirurgiei laparoscopice în patologia incriminat o fac net superioară metodei clasice ma ales prin caracterul minim invaziv şi scurtarea timpului operator şi implicit al celui anestezic. Beneficiile aduse de un minim protocol de includere pentru chirurgia laparoscopica la nivelul clinicii noastre au dus la rezultate postoperatorii superioare metodei laparoscopice şi cu un beneficiu evident asupra indicilor de spitalizare

Addressing gaps in care of people with conditions affecting sex development and maturation

Differences of sex development are conditions with discrepancies between chromosomal, gonadal and phenotypic sex. In congenital hypogonadotropic hypogonadism, a lack of gonadotropin activity results primarily in the absence of pubertal development with prenatal sex development being (almost) unaffected in most patients. To expedite progress in the care of people affected by differences of sex development and congenital hypogonadotropic hypogonadism, the European Union has funded a number of scientific networks. Two Actions of the Cooperation of Science and Technology (COST) programmes - DSDnet (BM1303) and GnRH Network (BM1105) - provided the framework for ground-breaking research and allowed the development of position papers on diagnostic procedures and special laboratory analyses as well as clinical management. Both Actions developed educational programmes to increase expertise and promote interest in this area of science and medicine. In this Perspective article, we discuss the success of the COST Actions DSDnet and GnRH Network and the European Reference Network for Rare Endocrine Conditions (Endo-ERN), and provide recommendations for future research

Long-Term Outcomes with Subcutaneous C1-Inhibitor Replacement Therapy for Prevention of Hereditary Angioedema Attacks

Background For the prevention of attacks of hereditary angioedema (HAE), the efficacy and safety of subcutaneous human C1-esterase inhibitor (C1-INH[SC]; HAEGARDA, CSL Behring) was established in the 16-week Clinical Study for Optimal Management of Preventing Angioedema with Low-Volume Subcutaneous C1-Inhibitor Replacement Therapy (COMPACT). Objective To assess the long-term safety, occurrence of angioedema attacks, and use of rescue medication with C1-INH(SC). Methods Open-label, randomized, parallel-arm extension of COMPACT across 11 countries. Patients with frequent angioedema attacks, either study treatment-naive or who had completed COMPACT, were randomly assigned (1:1) to 40 IU/kg or 60 IU/kg C1-INH(SC) twice per week, with conditional uptitration to optimize prophylaxis (ClinicalTrials.gov registration no. NCT02316353). Results A total of 126 patients with a monthly attack rate of 4.3 in 3 months before entry in COMPACT were enrolled and treated for a mean of 1.5 years; 44 patients (34.9%) had more than 2 years of exposure. Mean steady-state C1-INH functional activity increased to 66.6% with 60 IU/kg. Incidence of adverse events was low and similar in both dose groups (11.3 and 8.5 events per patient-year for 40 IU/kg and 60 IU/kg, respectively). For 40 IU/kg and 60 IU/kg, median annualized attack rates were 1.3 and 1.0, respectively, and median rescue medication use was 0.2 and 0.0 times per year, respectively. Of 23 patients receiving 60 IU/kg for more than 2 years, 19 (83%) were attack-free during months 25 to 30 of treatment. Conclusions In patients with frequent HAE attacks, long-term replacement therapy with C1-INH(SC) is safe and exhibits a substantial and sustained prophylactic effect, with the vast majority of patients becoming free from debilitating disease symptoms

Significant benefits of AIP testing and clinical screening in familial isolated and young-onset pituitary tumors

Context Germline mutations in the aryl hydrocarbon receptor-interacting protein (AIP) gene are responsible for a subset of familial isolated pituitary adenoma (FIPA) cases and sporadic pituitary neuroendocrine tumors (PitNETs). Objective To compare prospectively diagnosed AIP mutation-positive (AIPmut) PitNET patients with clinically presenting patients and to compare the clinical characteristics of AIPmut and AIPneg PitNET patients. Design 12-year prospective, observational study. Participants & Setting We studied probands and family members of FIPA kindreds and sporadic patients with disease onset ≤18 years or macroadenomas with onset ≤30 years (n = 1477). This was a collaborative study conducted at referral centers for pituitary diseases. Interventions & Outcome AIP testing and clinical screening for pituitary disease. Comparison of characteristics of prospectively diagnosed (n = 22) vs clinically presenting AIPmut PitNET patients (n = 145), and AIPmut (n = 167) vs AIPneg PitNET patients (n = 1310). Results Prospectively diagnosed AIPmut PitNET patients had smaller lesions with less suprasellar extension or cavernous sinus invasion and required fewer treatments with fewer operations and no radiotherapy compared with clinically presenting cases; there were fewer cases with active disease and hypopituitarism at last follow-up. When comparing AIPmut and AIPneg cases, AIPmut patients were more often males, younger, more often had GH excess, pituitary apoplexy, suprasellar extension, and more patients required multimodal therapy, including radiotherapy. AIPmut patients (n = 136) with GH excess were taller than AIPneg counterparts (n = 650). Conclusions Prospectively diagnosed AIPmut patients show better outcomes than clinically presenting cases, demonstrating the benefits of genetic and clinical screening. AIP-related pituitary disease has a wide spectrum ranging from aggressively growing lesions to stable or indolent disease course

Testosterone supplementation for hypogonadal men by the nasal route

© 2009 Informa plcAlthough multiple forms of testosterone replacement therapy are available to treat hypogonadism, none is ideal. This article reports on the pharmacokinetics of an innovative nasal formulation of testosterone in hypogonadal men. The first study was undertaken in eight men with a baseline total testosterone (TT) of 130.8 ± 87.4 ng/dL and examined the pharmacokinetics of nasal testosterone given in a single dose of 7.6 mg, 15.2 mg or 22.8 mg, respectively. The second study examined the pharmacokinetics of nasal testosterone (7.6 mg) given either twice or three times a day in 21 severely hypogonadal men (baseline TT in 20 patients <50 ng/dL, in one patient 152 ng/dL) for 14 days. The steady-state concentration of testosterone was within the normal range in all treatment groups, but only in the 3-times-a-day group was the 95% confidence interval completely within the physiological range. The average DHT level did not exceed the upper range of normal. The clinical global visual analogue scale improved in the whole group receiving testosterone (p < 0.001). All adverse events in both studies were of mild to moderate intensity and were evaluated as unlikely or not related to the administered study drug. No patients dropped out during treatment. Comparison with the normal circadian rhythm by computer modelling suggests that nasal testosterone can be used to mimic the normal diurnal pattern in eugonadal men. Thus, nasal testosterone can be administered safely to humans in doses that approximate serum concentrations in the normal physiological range.Claudia Mattern, Claudia Hoffmann, John E. Morley and Corin Badi