Cytoreductive treatment in patients with CALR-mutated essential thrombocythaemia: a study comparing indications and efficacy among genotypes from the Spanish Registry of Essential Thrombocythaemia.

The present study assessed the criteria for initiating cytoreduction and response to conventional therapies in 1446 patients with essential thrombocythemia (ET), 267 (17%) of which were CALR-mutated. In low risk patients, time from diagnosis to cytoreduction was shorter in CALR-positive than in the other genotypes (2·8, 3·2, 7·4 and 12·5 years for CALR, MPL, JAK2V617F and TN, respectively, P < 0·0001). A total of 1104 (76%) patients received cytoreductive treatment with hydroxycarbamide (HC) (n = 977), anagrelide (n = 113), or others (n = 14). The estimated cumulative rates of complete haematological response (CR) at 12 months were 40 % and 67% in CALR and JAK2V617F genotypes, respectively. Median time to CR was 192 days for JAK2V617F, 343 for TN, 433 for MPL, and 705 for CALR genotypes (P < 0·0001). Duration of CR was shorter in CALR-mutated ET than in the remaining patients (P = 0·003). In CALR-positive patients, HC and anagrelide had similar efficacy in terms of response rates and duration. CALR-mutated patients developed resistance/intolerance to HC more frequently (5%, 23%, 27% and 15% for JAK2V617F, CALR, MPL and TN, respectively; P < 0·0001). In conclusion, conventional cytoreductive agents are less effective in CALR-mutated ET, highlighting the need for new treatment modalities and redefinition of haematologic targets for patients with this genotype.

Role of therapeutic plasma exchanges in refractory severe warm autoimmune hemolytic anemia: Presentation of two case reports.

BACKGROUND: Warm autoimmune hemolytic anemia (WAIHA) is a disorder with a usually good response to corticosteroid treatment, whereas in some cases first-line treatment's response is poor and other therapies such as intravenous immunoglobulins (IVIGs), rituximab, or splenectomy must be applied. STUDY DESIGN AND METHODS: Herein, we describe two patients with severe WAIHA treated at our center, who obtained a response after therapeutic plasma exchanges (TPEs) combined with low doses of IVIG. RESULTS: The first patient was an 18-year-old man with no relevant past medical history who was diagnosed with WAIHA. The patient presented a progressive clinical worsening despite treatment with prednisone, IVIG, and rituximab. After starting TPEs, signs of hemolysis rapidly improved and hemoglobin started to recover. The second patient was a 38-year-old man with a past history of immune thrombocytopenia and WAIHA. The patient presented a new flare of WAIHA, with no response after 2 weeks of treatment with corticosteroids, IVIG, and rituximab. After initiation of TPEs, the patient had an improvement in hemolysis biomarkers and recovery of hemoglobin concentration. CONCLUSION: Combination of TPEs with rituximab and IVIG might be considered as a therapeutic option in patients with severe WAIHA without response to corticosteroid and IVIG treatment.

[Pre-operative symptoms and post-operative improvement in patients diagnosed with hyperparathyroidism. Analysis of 120 consecutive cases]. / Clínica preoperatoria y mejoría postoperatoria en pacientes con diagnóstico de hiperparatiroidismo. Análisis de 120 casos consecutivos.

OBJECTIVE: To compare the pre-operative and post-operative clinical symptoms in patients diagnosed as having hyperparathyroidism and given surgical treatment, in order to prove the existence of statistically significant improvement. MATERIAL AND METHOD: We report here a retrospective study performed on 120 consecutive patients operated on following diagnosis of hyperparathyroidism between 1990 and 2003. RESULTS: Nephrolithiasis, generalized bone pain and HBP were the most common clinical manifestations. Primary hyperparathyroidisms represented 76.7 %, while secondary ones were 20.8 % and 2.5 % were tertiary. We carried out 85 adenoma removals, 30 sub-total and 5 total parathyroidectomies. We only encountered one case of recurrent palsy and about 25 % of hypocalcemias (2 of them permanent). Osteoarticular pathology and nephrolithiasis suffered by our patients clearly improved after surgery (P< .01) after 2 years of follow-up. There was no significant improvement in HBP, digestive and psychiatric pathology or pruritus. CONCLUSIONS: The improvement in quality of life for most of the patients operated on for this condition amply justifies parathyroidectomy by an experienced otolaryngology team.

[Influence of non-neuronal factors on post-thyroidectomy dysphonia]. / Influencia de factores no neuronales en la disfonía tras tiroidectomía.

OBJECTIVES: We present a prospective study assessing the real incidence, aetiology, and evolution of vocal impairment following total thyroidectomy. PATIENTS AND METHOD: Sixty-six patients who underwent total thyroidectomy between 2003 and 2006 were included, recording demographic and analytic variables, with emphasis on subjective vocal changes observed after surgery, measured by means of the GRABS scale. A control group of 25 patients operated under general anaesthesia and oro-tracheal intubation was chosen using random sampling. RESULTS: In 30 patients (45.5 %), post-operative dysphonia was observed. Among these, only 2 (3 %) were permanent, and 11 were due to nerve damage (10 inferior and 1 superior laryngeal nerves). In 8 patients (12.2 %), GRABS score was above 5. In the rest of these 30 patients, the complaint was a single decreased tone (10 cases), lack of intensity (9 cases) and vocal fatigue (3 cases), always with a GRABS score equal to or less than 2 points. The most significantly related factor with the onset of dysphonia was the section of strap muscles (OR=12.5). CONCLUSIONS: Dysphonia is a common complication of total thyroidectomy, but its incidence is not always related to nerve injury. Some technical (and sometimes avoidable) factors, such as the section of prelaryngeal muscles, could have an important relationship with this adverse event.

[Acute epiglottitis in adults. Diagnosis and treatment in our experience of 30 cases]. / Epiglotitis aguda en adultos. Nuestra experiencia clínica en 30 casos.

OBJECTIVE: We present the 15-year-long experience of 2 hospitals in our region regarding the therapeutic management of acute epiglottitis in adults. PATIENTS AND METHOD: Thirty patients with an average age of 46 years were diagnosed as having acute epiglottitis, either by indirect laryngoscopy or fibroscopy, and studied through a series of clinical parameters: age, sex, personal history, complementary tests, clinical symptoms, treatment, evolution, and average stay in hospital. RESULTS: We found an obvious predominance of this urgent pathology in males, with most patients reporting dysphagia or odynophagia (90 %). Dyspnoea was confirmed in 40 % of the cases but only 7 required intubation, coniotomy, or tracheotomy. The complications recorded include one case of mediastinitis and another of death due to sudden cardiorespiratory arrest. CONCLUSIONS: We feel that a specific protocol must be established to take into account, apart from admission to hospital, personal contact with an intensive care unit (ICU) even though in most cases it is ultimately unnecessary to ensure airway patency as seen in the various case series published.

[Endoscopic treatment for choanal atresia]. / Tratamiento de la atresia de coanas por vía endoscópica.

Choanal atresia is an infrequent congenital malformation. Classically, 4 surgical approaches have been described for its treatment: transnasal, transpalatal, trans-septal, and transantral. Of these, transpalatal was preferred. In recent years, the progress in nasal endoscopy has led to reconsideration of the transnasal route as being less invasive and providing excellent results. The use of stents to prevent re-stenosis is a controversial issue. However, the application of substances such as mitomycin may offer decreased need for stenting by reducing the development of cicatrix tissue. We report the case of a patient with bilateral choanal atresia and its surgical treatment using nasal endoscopy with topical mitomycin. We report the case of a patient with bilateral choanal atresia and the surgical treatment with topical mitomycin.

[Surgical treatment of refractory hyperparathyroidism and usefulness of the intraoperative PTH]. / Tratamiento quirúrgico del hiperparatiroidismo refractario y utilidad de la paratirina intraoperatoria.

OBJECTIVE: To share our experience in the surgery of so-called refractory hyperparathyroidism (secondary and tertiary without response to therapy with calcitriol). MATERIAL AND METHODS: Retrospective study based on 41 patients-5 with secondary and 6 with tertiary hyperparathyroidism-referred by nephrology for surgical evaluation of their illness because of poor response to the medical treatment given. RESULTS: In 18 of the 41 cases we used the fast or turbo intra-operative PTH with reduction of more than 60 % in all patients. In the group in whom normal PTH was performed, we registered 2 secondary hyperparathyroidisms with no significant decrease and persistence of symptoms. One of them was reoperated successfully. DISCUSSION: Subtotal or total parathyroidectomy with reimplant represents the treatment of choice in refractory hyperparathyroidism with good results in most of the series reviewed.

[Contralateral hemithyroidectomy due to carcinoma of the thyroid. Our cases reviewed and updated]. / Hemitiroidectomía contralateral por carcinoma de tiroides. Nuestra casuística revisada y actualizada.

OBJECTIVE: We are reporting our 14 years' experience with prior hemithyroidectomies or contralateral hemithyroidectomies after a pathology result reporting malignancy (thyroid carcinoma) in the first surgery. MATERIAL AND METHOD: Twenty female patients with an average age of 45 years old have been studied and we have analyzed the initial symptoms, results of complementary tests, pathology diagnosis following initial surgery, and final outcome after a second intervention. RESULTS: The incidence of malignancy shown in our series after secondary surgery was 40 % and the percentage of hemithyroidectomy on hemithyroidectomy was 3 % after operating on 650 thyroid glands. CONCLUSIONS: Though opinions differ in the medical literature, we feel a total thyroidectomy must be performed after a casual finding of thyroid carcinoma because of the oncologically safer outcome and the better control of the patient.

Frecuencia del manejo quirúrgico con malla de la incontinencia urinaria de esfuerzo en el Hospital Nacional Arzobispo Loayza / Frequency mesh surgical management of urinary incontinence in the Arzobispo Loayza National Hospital

Objetivo: Determinar la frecuencia del manejo quirúrgico con malla de la incontinencia urinaria de esfuerzo en el HNAL durante el año 2012. Material y Método: Estudio observacional, descriptivo y retrospectivo. La población incluyó todas las mujeres diagnosticadas con IUE y que recibieron tratamiento quirúrgico en el Servicio de Ginecología del HN A. Loayza con registro operatorio completo en el libro de Sala de Operaciones. Se llenó una ficha de recolección de datos, se creó una base de datos y se realizó el análisis con el programa estadístico Excel. Resultados: 60 pacientes fueron sometidas a cirugía por IUE, con o sin distopia genital agregada y con patologías subyacentes. El promedio de edad de las pacientes fue de 59 años. El uso de mallas fue del 73,33% de los procedimientos realizados. Conclusiones: La técnicas de suspensión uretral sin tensión con abordaje por vía vaginal fue la de mayor uso en el Hospital Loayza, con la ventaja que se puede combinar con otras cirugías del tracto genital como histerectomía abdominal o vaginal y cura quirúrgica de cistocele.

Objective: Determine the frequency of Mesh Surgical Management of Urinary Incontinence in the HNAL during 2012. Material and Methods: Observational, descriptive and retrospective study. The population includes all women diagnosed with SUI and underwent surgery in the Department of Gynecology A. Loayza NH full operative registration book Operating Room. A form of data collection was filled, created a database and statistical analysis with the Excel program was performed. Results: 60 patients underwent surgery for SUI with or without genital dystopia aggregate and underlying pathologies. The average patient age was 59 years. The use of Mesh was 73.33% of the procedures performed. Conclusions: The techniques tension-free urethral suspension vaginal approach was the most used in the Loayza Hospital, with the advantage that can be combined with other genital tract surgeries like abdominal or vaginal hysterectomy and surgical cure of cystocele.