[Erythema nodosum during the course of idiopathic granulomatous mastitis]. / Érythème noueux au cours d'une mastite granulomateuse idiopathique.

BACKGROUND: Idiopathic granulomatous mastitis (IGM) is a benign, aseptic inflammatory disease of unknown origin, which must be distinguished from tumoral and infectious processes that affect the breast, including tuberculosis. IGM is a rare cause of erythema nodosum, but it is useful for dermatologists to be aware of this association. PATIENTS AND METHODS: A 32-year-old nulliparous woman presented with erythema nodosum, arthralgia and fever. On examination, she had a firm and painful mass of 5cm in the right breast with retraction and axillary adenopathy. The breast lump developed gradually over the preceding 4 months. Although two biopsies showed no evidence of atypical cells, inflammatory areas and a granulomatous process were seen. Culture of breast tissue for mycobacteria was negative. A diagnostic of idiopathic granulomatous mastitis was made. Systemic corticosteroids led to a reduction in size of the mass, but relapse occurred in the contralateral breast on dose-reduction of the corticosteroids. DISCUSSION: IGM is a rare disease of unknown aetiology. Diagnosis is based on characteristic histological features and exclusion of other granulomatous diseases. Extra-mammary signs are rare and include erythema nodosum, arthralgia and episcleritis. Management is poorly codified.

[Neutrophilic sebaceous adenitis in a woman]. / Adénite sébacée neutrophilique chez une femme.

BACKGROUND: Neutrophilic sebaceous adenitis is a very rare disease with only three reported cases, all involving men. Herein, we describe the first case in a woman. MATERIALS AND METHODS: A 25-year-old woman presented erythematous and indurated circinate plaques on the face, upper chest and upper limbs, associated with some pustules. The eruption started immediately after sunny holidays and extended progressively with low-grade fever and axillar polyadenopathies. Blood tests showed moderately elevated neutrophils and elevated erythrocyte sedimentation rate. Cutaneous biopsy showed an inflammatory infiltrate composed of lymphocytes, histiocytes and neutrophils with perisebaceous distribution, infiltrating sebaceous glands with focal necrosis of sebocytes. Treatment consisting of topical steroid and photoprotection quickly resulted in regression of the lesions, with no relapse after two months. DISCUSSION: Classically neutrophilic sebaceous adenitis presents as erythematous and violaceous, indurated, circinate plaques with raised edges on the face and upper chest. Spontaneous regression is frequently reported. Histological examination is typical with an inflammatory infiltrate containing neutrophils with primarily perisebaceous distribution and penetrating sebaceous glands in places with necrosis of sebocytes. The aetiology is unknown but in a recent case, photodermatosis was suspected because lesions occurred every summer. This hypothesis is consistent with our observation but the presence of lesions on non-exposed areas raises questions about heat as a possible trigger factor in this disorder.

Effect of infection control strategy on knowledge, attitude and practice towards hepatitis B transmission and prevention in vulnerable populations.

BACKGROUND: Health care workers (HCWs) and hematological patients needing blood/ blood product transfusion are particularly vulnerable to blood born infections (BBI) including viral hepatitis. OBJECTIVE: To evaluate knowledge, attitude and practice (KAP) of these target groups regarding viral hepatitis B (HBV) transmission and its change with implementing infection control policy and procedures. METHODS: An anonymous questionnaire with closed questions was used to evaluate KAP including vaccination status in 2 target groups, in Children Hospital, Ain Shams University, Cairo, Egypt: 184 nurses and 210 children and adolescents with blood diseases. One year after instituting infection control as a part of hospital procedures, the same questionnaire was reused to evaluate KAP towards HBV. RESULTS: Baseline knowledge regarding HBV transmission, sequelae and preventive measures, was poor in both groups. Among nurses, only 62% wore gloves on withdrawing or giving blood to patients, 43.5% routinely washed hands between patients and 37.5% reported exposure after sharp injury. Only 38% of patients and 40% of nurses received HBV vaccination. Targeted infection control policy and procedures significantly improved KAP regarding HBV in both groups. Vaccination coverage significantly increased and reached 88.7% for nurses and 72% for patients. CONCLUSIONS: Hospital based infection control units with established policy and procedures against BBI significantly improved KAP towards HBV including a significant increase in vaccination intake.

Perioperative variables associated with surgical site infection in breast cancer surgery.

Despite the fact that breast operations are usually categorized as clean procedures, higher surgical site infection (SSI) rates are reported. This study aimed to determine the perioperative variables related to SSI in breast cancer patients. Medical records of breast cancer patients undergoing surgery between January 2005 and August 2007 at a university based hospital were reviewed. Preoperative, intraoperative, and postoperative clinical data from 199 patients were extracted and analysed. Overall, the SSI rate was 19.1% (38 cases). SSI was associated with a high body mass index (P=0.001), history of diabetes mellitus (P<0.0001), smoking (P<0.0001), or active skin disorders (P<0.0001). Other SSI-related variables included a tumour at an advanced clinical stage (P=0.003) and neoadjuvant therapy (P=0.003). Breast-conserving operations were less frequently associated with SSI than were radical procedures (mastectomy alone and mastectomy followed by immediate reconstruction) (P=0.0001).

Presentación inicial de las pacientes con diagnóstico de cáncer de seno en el Centro Javeriano de Oncología, Hospital Universitario San Ignacio / Initial presentation of patients with diagnosis of breast cancer at the Centro Javeriano de Oncología of Hospital Universitario San Ignacio

Introducción. El cáncer de seno es un problema de salud pública en Colombia que amerita atención por su incidencia y crecientes tasas de mortalidad. Las características de su presentación inicial son importantes, puesto que determinan el estadio clínico y, por consiguiente, el pronóstico de la enfermedad; por lo tanto, es importante conocer cómo llegan las pacientes con cáncer de seno a su primera consulta en nuestra institución.Objetivo. Describir las características demográficas y clínicas de las mujeres con diagnóstico de cáncer de seno en el Centro Javeriano de Oncología, Hospital Universitario San Ignacio.Materiales y métodos. Estudio observacional descriptivo-retrospectivo de 232 casos consecutivos, de pacientes diagnósticados por primera vez con cáncer de seno, de los cuales se obtuvieron los siguientes datos: edad, motivo de consulta, enfermedad actual, antecedentes relevantes, examen clínico de seno, diagnóstico histológico y estadificación clínica.Resultados. Se encontró una tendencia hacia una población mayor de 50 años (63,4 por ciento), que consultó más frecuentemente por presencia de masa (77 por ciento) y predominio de tumores ductales (91 por ciento). Los estadios clínicos más frecuentes fueron IIIB (25 por ciento), IIA (17 por ciento), IIB (16 por ciento) y I (16 por ciento).Conclusión. En el periodo de estudio, los nuevos casos de carcinoma mamario se presentaron en su mayoría en estadios temprano y localmente avanzado, distribución semejante a la reportada por otros grupos en Bogotá, Colombia.

Introduction. Breast cancer is a public health issue in Colombia that deserves awareness due to its increasing incidence and mortality rates. Initial presentation characteristics are important because these determine the clinical stage, and consequently the prognosis. It is important to understand how women with breast cancer are presenting at our center.Objective. To describe the demographic and clinical characteristics of women with breast cancer diagnosed at Centro Javeriano de Oncología, Hospital Universitario San Ignacio (HUSI).Methods. Retrospective, descriptive observational study of 232 consecutive, newly diagnosed breast cancer cases of which the following data was obtained from clinical charts: age, chief complaint, medical history, clinical breast exam, histologic diagnosis, and clinical staging.Results. The population tended to be over 50 years (63.4 percent), generally referred because of the presence of a lump (77 percent); tumors were predominantly ductal, in 91 percent of the cases. The most frequent clinical stages were IIIB (25 percent), IIA (17 percent), IIB (16 percent), and y I (16 percent).Conclusion. In this study, newly diagnosed breast cancer cases mainly presented in early and locally advanced stages, a distribution similar to that reported by other research groups in Bogotá, Colombia.

Cutaneous manifestations of immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome.

BACKGROUND: Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is a rare disorder characterized by neonatal autoimmune enteropathy, diabetes and thyroiditis, food allergies and skin rash. IPEX syndrome is caused by mutations in FOXP3, a master control gene of regulatory T cells (Tregs), resulting in absent or dysfunctional Tregs. Data in the literature are scarce and the cutaneous manifestations are rarely depicted. OBJECTIVES: To evaluate the frequency and characteristics of cutaneous manifestations found in IPEX. METHODS: Retrospective single-centre study of a case series of IPEX. Patients' data were retrieved from medical files and numerous parameters concerning general and cutaneous characteristics of the disease were recorded. RESULTS: Ten children with IPEX were studied. Cutaneous involvement was present in seven of 10 children; age at onset was 0-4 months, median 1.5. All patients presented with atopic dermatitis (AD). Three presented more psoriasiform lesions. Eczema was severe; most affected areas were lower limbs, trunk and face. Pruritus was present in four of seven, and painful fissurary cheilitis in four of seven. Hyper-IgE was found in seven of 10 and hypereosinophilia in five of 10. Skin biopsies showed eczematiform or psoriasiform features. Affected patients were improved by dermocorticoids; no clear improvement was obtained with immunosuppressive regimens. Other features were urticaria secondary to food allergies and staphylococcal sepsis, mostly Staphylococcus aureus and catheter related. CONCLUSIONS: AD seems to be a frequent finding in IPEX syndrome, which is characterized by Treg anomalies. This hints to a possible role of Tregs in AD, which is then discussed in this study.

Sickle cell leg ulcers: a frequently disabling complication and a marker of severity.

BACKGROUND: Leg ulcers are a poorly known and underestimated complication of sickle cell disease (SCD), but in our experience they often appear as a severely disabling condition, associated with the most severe forms of the disease. OBJECTIVES: To assess the characteristics, complications, repercussion on quality of life and associations of SCD ulcers. METHODS: Case series of 20 patients followed in a French referral centre for SCD and who had previous/present leg ulcers. RESULTS: Median ulcerated area was 12 cm2 and median time spent with ulcers was 29.5 months. Locoregional infections developed in 85%, ankle stiffness in 50% and mood disorders in 85%. Ninety per cent of patients needed analgesics, 20% being opioids. Median loss of time from work was 12.5 months. The Short Form 36 Health Survey showed physical and mental component summary scores of 41.5 and 40.7, respectively, indicating severe alteration close to that found in lung cancer or haemodialysis. Two categories of SCD leg ulcers were distinguished, defined by a 1-year duration cut off. The 'prolonged' ulcers had larger surfaces, tended to recur more frequently and led to more infection and depression. Several SCD complications were associated with leg ulcers, notably priapism, pulmonary hypertension, stroke and acute chest syndrome. CONCLUSIONS: Leg ulcers are a major complication of SCD, given their severe consequences and frequent association with other specific organ damage, and they constitute in their 'prolonged' form a severely disabling condition that remains an important therapeutic challenge.

Abnormal glucose tolerance in Egyptian beta-thalassemic patients: possible association with genotyping.

BACKGROUND: Type 1 diabetes mellitus (DM) is a frequent complication in patients with beta-thalassemia. It is believed to be due to the damage inflicted by iron overload of the pancreatic beta cells. Liver disorders and genetic influences seem to be additional predisposing factors. OBJECTIVE: To study the prevalence of diabetes and impaired glucose tolerance (IGT) in transfusion-dependent Egyptian beta-thalassemic patients and to evaluate the possible role of genotyping in the pathogenesis of diabetes associated with beta-thalassemia. RESEARCH DESIGN AND METHODS: A total of 56 transfusion-dependent beta-thalassemic patients aged 10-31 (mean age=15.9 +/- 5.7 yr), 32 males and 24 females, including 48 thalassemia major and eight thalassemia intermedia; compared to 15 age- and sex-matched controls. All were subjected to history and examination, laboratory investigations: complete blood count (CBC), serum ferritin, liver function tests, hepatitis B and C markers, fasting blood glucose, oral glucose tolerance test (OGTT) and fasting C-peptide. Genotyping for 16 mutations was assessed in thalassemic patients with abnormal glucose tolerance. RESULTS: The prevalence of diabetes was 10.4% (5 of 48) and IGT was 14.6% (7 of 48) among thalassemia major, whereas, none of thalassemia intermedia had abnormal glucose tolerance. Fasting C-peptide was lower in beta-thalassemic patients compared to controls (p <0.001); the level was significantly higher in patients complicated by diabetes or IGT compared with other thalassemic patients (p <0.001). Chronic hepatitis C was detected in all patients (100%) with abnormal glucose tolerance. Genotyping showed that IVS II nt 745 was detected in 77.7% of cases with abnormal glucose tolerance. CONCLUSIONS: Abnormal glucose tolerance is common in multiply transfused beta-thalassemia major patients, which could be attributed to progressive and early loss of beta-cell mass, along with persistent insulin resistance. Chronic hepatitis C may play a role in the development of abnormal glucose tolerance. An association between diabetes and genotyping IVS II nt 745 was found. Patients with this particular genotype are advised to check their blood glucose every 6 months to detect early occurrence of diabetes.

Conceptos actuales sobre ganglio centinela en cáncer mamario / Current concepts on sentinel lymph node in mammary cancer

El concepto de ganglio centinela pretende obtener la misma informacion pronostica que ofrece el vaciamiento ganglionar acerca del compromiso metastásico axilar, con la ventaja de que disminuye cuantitativamente la considerable morbilidad relacionada con la diseccion de los niveles I y II. Existen considerables incentivos para aceptar la biopsia del ganglio centinela sin diseccion axilar como una práctica estandar. Sin embargo, la diseccion axilar clasica no debe ser abandonada hasta que se documente una extensa experiencia y bajas tasas de falsos negativos con la nueva tecnica en manos de un mismo cirujano y dentro de un mismo grupo hospitalario.

Duplicación gástrica / Gastric duplication

La duplicacion gastrica (DG) es una entidad congenita muy rara que acontece en el 3.8 por ciento de las duplicaciones entericas y se diagnostica generalmente en los primeros años de vida. Hasta ahora solo hay 20 casos en adultos, informados en la literatura. En este trabajo se informa el caso de duplicacion gastrica en un paciente de 57 años, con sintomas digestivos altos de varios años de evolucion, a quien se le detecta por ecografia y tomografia computarizada (CT) una lesion quistica en relacion con la cola del pancreas, la pared posterior del estomago y el bazo, se encontro en la laparotomia exploratoria una masa hacia la curvatura mayor del estomago, la cual se reseco y su histologia informo una duplicacion gastrica completa.

Asymptomatic carotid artery stenosis and stroke in patients undergoing cardiopulmonary bypass.

PURPOSE: This study was undertaken to assess the natural history of carotid artery stenosis in patients undergoing cardiopulmonary bypass (CPB) at a Veterans Administration Medical Center. METHODS: Between January 1989 and August 1993, all patients undergoing CPB were offered preoperative carotid artery ultrasound screening as part of an investigative protocol. Patients were monitored in-hospital for the occurrence of perioperative neurologic deficit. RESULTS: A total of 582 patients underwent carotid artery ultrasound screening. Greater than 50% stenosis or occlusion of one or both internal carotid arteries was present in 130 patients (22%), with 80% or greater stenosis or occlusion of one or both arteries present in 70 patients (12%). In-hospital stroke or death occurred in 12 (2.1%) and 36 (6.2%) patients, respectively. Of the 12 strokes, five were global and seven were hemispheric in distribution. Of the five patients who had global events, none had evidence of carotid artery stenosis. However, of the seven patients who had hemispheric events, five had significant 50% or greater stenosis or occlusion of the internal carotid artery ipsilateral to the hemispheric stroke. Therefore the presence of carotid artery stenosis or occlusion was significantly associated with hemispheric stroke (no stenosis 0.34% vs stenosis 3.8%; p = 0.0072). Furthermore, the risk of hemispheric stroke in patients with unilateral 80% to 99% stenosis, bilateral 50% to 99% stenosis, or unilateral occlusion with contralateral 50% or greater stenosis was 5.3% (4 of 75). No strokes occurred in patients with unilateral 50% to 79% stenosis (n = 52). CONCLUSIONS: It is concluded that carotid atherosclerosis is a risk factor for hemispheric stroke in patients undergoing CPB.

Autonomously functioning thyroid nodules in childhood and adolescence.

Autonomously functioning thyroid nodules (AFTNs) in children and adolescents (under age 18) are unusual but are not as rare as earlier reports suggested. These lesions have a significantly different biologic potential than similar lesions in older patients. In the younger age group there is a more rapid progression toward toxicity and a higher incidence of thyroid carcinoma. Our experience with 12 patients is combined with those previously reported for identification of a total of 61 children and adolescents with AFTNs, of whom 53 have undergone operation. Hyperthyroidism was present in 15 patients (24.6%), and in six patients (11.3%) the AFTN was due to a well-differentiated thyroid carcinoma. Surgical treatment is advisable for all children and adolescents with AFTNs because of the risks of hyperthyroidism and thyroid carcinoma. Surgical excision (lobectomy is preferred) results in rapid restoration of a euthyroid state for the toxic AFTN and allows histopathologic diagnosis. Therapy with radioiodine is not advisable for treatment of AFTNs in this age group. Thyroid-stimulating hormone suppression should be used for all patients with a diagnosis of thyroid carcinoma.

Penetrating wounds of the back and flank: analysis of 77 cases.

To determine the factors that allow early recognition of injuries to the intrathoracic and intra-abdominal organs in patients with penetrating wounds to the lower thorax and abdomen with site of entrance located in the back or flanks, we retrospectively analyzed the cases of 77 such patients. There were 65 stab wounds (85%) and 12 gunshot wounds (15%). The injuries were confined to the abdomen in 46 (60%); 39 were stab wounds and seven were gunshot wounds. The wounds were confined to the chest in 24 patients (31%) and involved both the chest and abdomen in seven cases (9%). Of the 53 abdominal wounds, 21 necessitated celiotomy; in 20 of them, an intra-abdominal organ was found injured, and one patient died (1.9%). Of the 24 injuries confined to the chest, only one patient required emergency thoracotomy due to massive hemorrhage; all 24 patients survived. All of the seven thoracoabdominal injuries, two produced by gunshot and five by stabbing, necessitated celiotomy. Most patients with gunshot wounds to the back and flanks require celiotomy, but a selective therapy can be safely used in patients with stab wounds. The rate of negative exploratory celiotomy was less than 5%; the mortality was 1.3%.