Sickle Cell Disease in Jordan: The Experience of a Major Referral Center.

INTRODUCTION: Sickle cell disorders are the most frequently encountered hemoglobin variants in Jordan. Both alpha and beta thalassemias are also prevalent in this population. However, studies on the interaction between these hemoglobin disorders are lacking. AIM: To determine the genotypes responsible for Sickle cell disease in Jordan, by retrospectively reviewing the data from a major referral center in the country's capital. METHODS: A total 29,712 peripheral blood samples referred and investigated for hemoglobinopathies over a 10-year period at Princess Iman Center at Amman, Jordan were retrospectively reviewed. In addition to full blood counts, high performance liquid chromatography, those who were identified with sickle cell hemoglobin were studied using polymerase chain reaction and reverse hybridization to determine the various sickle cell disease genotypes. RESULTS: Out of the (29,712) blood samples, 450 were sickle cell trait, while 216 had sickle cell disease. Of the latter: 120 were found to be cases of Sickle cell anemia (Hb SS), 66 were compound heterozygous for Sickle cell and a beta thalassemia mutation (Sickle/ß-thalassemia), while 30 had concomitant alpha thalassemia (HbSS/alpha thalassemia). The most frequent genotype associated with sickle/ß-thalassemia was HbS/ IVS-110 (G>A), followed by Hb S/IVS-I-6 (T>C), HbS/IVS-II-745 (C>G) and HbS/ IVS-II-1 (G>A). While the most frequent alpha genotype detected in HbSS/α-thalassemia samples was (-α3.7/αα) followed by (-α3.7/-α3.7). Hb SS patients had the severest hematological phenotype compared to those with sickle/ß-thalassemia and sickle/ α-thalassemia. Furthermore, within the sickle/ß-thalassemia subgroup the least severe hematological phenotype was encountered in HbS/IVS-1-6 (T>C), while the most severe in HbS/IVS-II-1 (G>A) genotype. CONCLUSION: The most frequent Sickle cell disease genotype in Jordanians is Sickle cell anemia (HbSS), followed by Sickle/ß-thalassemia and least frequent is HbSS/alpha thalassemia. The concomitant identified thalassemia mutations were consistent with their spectrum among the Jordanian population.

Attrition during pre-ART and ART time periods among adolescents enrolled in Integrated HIV Care Programme in Myanmar, 2005-2017.

Retaining adolescents (aged 10-19 years), living with HIV (ALHIV) on antiretroviral therapy (ART) is challenging. In Myanmar, 1269 ALHIV were under an Integrated HIV Care (IHC) Programme by June 2017 and their attrition (death and lost to follow-up) rates were not assessed before. We undertook a cohort study using routinely collected data of ALHIV enrolled into HIV care from July 2005 to June 2017 and assessed their attrition rates in June 2018 by time-to-event analysis. Of 1269 enrolled, 197(16%) and of 1054 initiated ART, 224 (21%) had an attrition defining event. The pre-ART and ART attrition rates were 21.8 (95% CI 19.0-25.1) and 6.4 (95% CI 5.6-7.3) per 100 person-years follow-up, respectively. The factors 'at enrolment' that were associated with higher hazards of attrition were: (1) WHO stage 3 or 4; (2) haemoglobin <10 gm/dl; (3) no documented CD4 cell counts, hepatitis B and C test results; and (4) injection drug use. Baseline hazards were high during the initial 1-2 years and after 5-6 years. The pre-ART and ART attrition rates in ALHIV were lower than those in Africa but higher than the children under IHC. This warrants designing and implementing additional care tailored to the needs of ALHIV under IHC.

Brain-derived neurotrophic factor genotype is associated with brain gray and white matter tissue volumes recovery in abstinent alcohol-dependent individuals.

Neuroimaging studies have linked the methionine (Met) allele of the brain-derived neurotrophic factor (BDNF) gene to abnormal regional brain volumes in several psychiatric and neurodegenerative diseases. However, no neuroimaging studies assessed the effects of this allele on brain morphology in alcohol use disorders and its demonstrated change during abstinence from alcohol. Here we assessed the effects of the BDNF Val66Met (rs6265) polymorphism on regional brain tissue volumes and their recovery during short-term abstinence in treatment-seeking alcohol-dependent individuals. 3D T1 weighted magnetic resonance images from 62 individuals were acquired at 1.5 T at one week of abstinence from alcohol; 41 of the participants were rescanned at 5 weeks of abstinence. The images were segmented into gray matter (GM), white matter (WM) and cerebrospinal fluid and parcellated into regional volumes. The BDNF genotype was determined from blood samples using the TaqMan technique. Alcohol-dependent Val (Valine)/Met heterozygotes and Val homozygotes had similar regional brain volumes at either time point. However, Val homozygotes had significant GM volume increases, while Val/Met heterozygotes increased predominantly in WM volumes over the scan interval. Longitudinal increases in GM but not WM volumes were related to improvements in neurocognitive measures during abstinence. The findings suggest that functionally significant brain tissue volume recovery during abstinence from alcohol is influenced by BDNF genotype.

Atención a los efectos secundarios de la radioterapia / Management of side effects of radiotherapy

Más del 50 per cent de pacientes con cáncer precisarán tratamiento con radioterapia para el control tumoral o como terapia paliativa. Pese a ello la radioterapia y sus efectos secundarios son poco conocidos para la mayoría de los profesionales de la Atención Primaria. Los efectos secundarios pueden ser agudos o crónicos y están relacionados con la dosis y su fraccionamiento, con el tamaño del tumor y extensión, volumen de tejido normal irradiado, tratamientos concomitantes y variables individuales del paciente. El objetivo de este artículo es informar al profesional de Atención Primaria de los principales efectos secundarios y su manejo (AU)

[The mission]. / La misión (y III).

After having made a historical review of the concept of mission statement, of evaluating its importance (See Part I), of describing the bases to create a mission statement from a strategic perspective and of analyzing the advantages of this concept, probably more important as a business policy (See Parts I and II), the authors proceed to analyze the mission statement in health organizations. Due to the fact that a mission statement is lacking in the majority of health organizations, the strategy of health organizations are not exactly favored; as a consequence, neither are its competitive advantage nor the development of its essential competencies. After presenting a series of mission statements corresponding to Anglo-Saxon health organizations, the authors highlight two mission statements corresponding to our social context. The article finishes by suggesting an adequate sequence for developing a mission statement in those health organizations having a strategic sense.

[Professional development: the mission (II)]. / Desarrollo profesional. La misión (II).

In the second part of this generic article whose purpose is to analyze what is a mission statement of an organization, the author concentrates on two fundamental points: what are the bases upon which a mission statement is constructed from a strategic perspective and what are the functions and advantages of a mission statement. With respect to this first basis, the author points out that an organization's function should be based on sound business criteria; that a mission statement should harmonize with the organizational milieu, reflect the values of the organization's professional staff while paying attention to special interest groups; and focus on professional know-how and on what works well and makes sense for the client. Concerning this second aspect, this article deals with maintaining the integrity of an organization, motivating professionals or utilizing a mission statement as a means of control.

Lost stones during laparoscopic cholecystectomy.

BACKGROUND: Gallbladder perforation, with loss of calculi in the abdomen is frequent during laparoscopic cholecystectomy. Recent publications report complications in port sites or in the abdominal cavity. A study of 3686 laparsocopic cholecystectomies performed by 6 surgeons was undertaken. In 627 patients, perforation of the gallbladder occurred and in 254 stones were spilled into the abdominal cavity. In 214 they were retrieved and in 40 left in the abdomen. Twelve patients developed complications. Percutaneous drainage was successful in 2 with serous collections. Two patients with abdominal abscesses were reoperated, stones retrieved and the abdomen drained. One patient developed an intestinal obstruction due to a stone in the ileum. One patient who had a cholecystectomy in another hospital developed a paraumbilical tumor. At reoperation a stone was retrieved. In another six patients, stones were found in port sites. Stones lost into the abdomen should be removed because of their potential morbidity, especially if they are large or if infection is present in the gallbladder at the time of initial surgery. There is no indication for routine conversion to open surgery when stone spillage occurs, although patients should be informed to avoid legal consequence, and to hasten early diagnosis of later complications.

Hepatic infarction in a pregnant patient with the 'primary' antiphospholipid syndrome.

We describe a patient with previous venous thrombosis while using oral contraceptives and recurrent pregnancy loss, who presented with massive hepatic infarction in the last trimester of the fourth gestation. Thrombocytopenia, the lupus anticoagulant (LA) and the anticardiolipin antibody (aCL) were detected and a diagnosis of a 'primary' antiphospholipid syndrome (APS) was made. The clinical and histological manifestations and the differential diagnosis, especially with DIC and pre-eclampsia, are discussed.

[Q fever on the island of La Palma. A review of 35 patients]. / Fiebre Q en la isla de La Palma. Revisión de 35 pacientes.

We present 35 patients diagnosed as suffering from Q fever during 1986, 1987, 1988. We establish a diagnosis criteria of complement titres greater than or equal to 1/64 or indirect immunofluorescent titres (IIT) 4 or more times higher than the initial, or a maintained title greater than or equal to 1/640 with specific IgM detected by positive IIT. 80% were male with a median age of 40.3 years. 85% had had contact with goats. 77% lived on the west side of the island 88.5% had increased GOT and GPT and only 5.7% had pneumonia. One patient previously healthy had ards, needing prolonged mechanical ventilation, and one patient had A-V blockade grade III which was treated successfully. We comment on the high incidence observed, which showed and endemic situation, and the epidemiological and clinical features of the disease.

Cancer del munon gastrico. Secuela alejada de las gastrectomia por ulcera? A proposito de 18 casos. / Cancer of the gastric stump. Delayed sequela of gastectomy for ulcer? Apropos of 18 cases

Se presentan 18 casos de cancer en el munon de pacientes sometidos a gastrectomias 15 por ulcera duodenal y 3 gastricas, que se presentaron a un promedio de 20 anos despues de la operacion. Siete casos fueron resecados; ninguno curo. La bibliografia pareceria demostrar la conveniencia del seguimiento alejado en todo gastrectomizado y de la investigacion del "cambio de habito" digestivo que puede preceder a la sintomatologia tardia asi como la necesidad de un mejor conocimiento de esta afeccion

Cancer del munon gastrico. Secuela alejada de las gastrectomia por ulcera? A proposito de 18 casos. / Cancer of the gastric stump. Delayed sequela of gastectomy for ulcer? Apropos of 18 cases

Se presentan 18 casos de cancer en el munon de pacientes sometidos a gastrectomias 15 por ulcera duodenal y 3 gastricas, que se presentaron a un promedio de 20 anos despues de la operacion. Siete casos fueron resecados; ninguno curo. La bibliografia pareceria demostrar la conveniencia del seguimiento alejado en todo gastrectomizado y de la investigacion del "cambio de habito" digestivo que puede preceder a la sintomatologia tardia asi como la necesidad de un mejor conocimiento de esta afeccion