Dizygotic twins with prolonged jaundice and microcytic, hypochromic, hemolytic anemia with pyropoikilocytosis.

Dizygotic twin males, born at 34 weeks gestation, had prolonged jaundice, microcytic, hypochromic anemia, FABarts hemoglobin, elevated end-tidal CO, and blood films consistent with hereditary pyropoikilocytosis. DNA sequencing revealed both had a heterozygous alpha spectrin (SPTA1) mutation (c.460_462dup) inherited from their asymptomatic mother, plus a 3-base pair duplication in alpha globin (HBA2) (c.364_366dupGTG) inherited from their asymptomatic father.

The color of skin: green diseases of the skin, nails, and mucosa.

Greenish staining of human skin may result from a gamut of causes, such as chlorosis, exogenous copper, resolving ecchymosis, drugs, green textile dyes, green tattoos, apocrine and eccrine chromhidrosis, hyper biliverdinemia, chloromas, use of green dyes during tube feeding in patient with multiorgan failure, Pseudomonas infections, and Wells' syndrome in its second stage. Physicians may rarely encounter patients with green skin, hair, nails, or mucosae.

Segurança do paciente: alterações da hemoglobina e ferro sérico após lipoaspiração e/ou abdominoplastia / Patient safety: changes in hemoglobin and serum iron after liposuction and/or abdominoplasty

Introdução: A lipoaspiração é o sendo segundo procedimento mais realizado no Brasil. Com os avanços da técnica, melhores equipamentos e aumento da segurança no ambiente cirúrgico, passaram-se a ser lipoaspiradas grandes áreas. Contudo, existe uma preocupação com a segurança do paciente. Comitês de segurança têm feito recomendações sobre a infiltração, anestesia, seleção do paciente, volume aspirado, entre outros. A Sociedade Brasileira de Cirurgia Plástica e o Conselho Federal de Medicina também determinaram parâmetros de volumes de segurança do aspirado e superfície corporal aspirada. Ainda assim, a literatura de apoio para as recomendações é escassa. O objetivo é avaliar as alterações dos níveis de hemoglobina, ferro sérico e proteínas totais no pré e pós-operatório mediato (7-10 dias), assim como seus reflexos nos pacientes. Métodos: Realizamos um estudo prospectivo, com pacientes submetidos à lipoaspiração isolada e associada à dermolipectomia abdominal, por indicações estéticas. Avaliamos 30 pacientes, sendo coletados dados demográficos, peso, Índice de Massa Corporal, níveis de hemoglobina, ferro sérico e proteínas totais, no pré-operatório, e entre 7 a 10 dias de pós-operatório. Resultados: A queda da hemoglobina foi entre 2-6g/dl, com média de 3,1g/dl aos 7-10 dia de PO. Tivemos como valor mínimo de hemoglobina 7,8g/dl no pós-operatório com 7-10 dias, enquanto o ferro sérico apresentou redução média de 44,87g/dl. Conclusão: A redução no nível da hemoblogina foi responsável pela sintomatolgia clinica apresentada. A reposição de ferro se mostrou necessária no pós-operatório imediato.

Introduction: Liposuction is the second commonest procedure performed in Brazil. The scope for liposuction has broadened with advances in the technique, with better equipment, and increased safety in the surgical environment. However, there are concerns about patient safety. Safety committees have made recommendations on various aspects of the procedure including infiltration, anesthesia, patient selection, and aspirated volume. The Brazilian Society of Plastic Surgery and the Federal Council of Medicine have also determined the parameters for aspiration safety volumes, and for the aspiration of body surface. However, supporting literature for the recommendations is scarce. The objective is to evaluate changes in hemoglobin, serum iron, and total protein levels in the peri-operative days (between 7 and 10 days), in addition to its impact in patients. Methods: We performed a prospective study, with patients submitted to either liposuction alone, or in conjunction with abdominal dermolipectomy for esthetic indications. We evaluated 30 patients, collecting their demographic data, weight, body mass index, hemoglobin, serum iron, and total protein levels in both, the pre-operative period, and between 7 and 10 post-operative days. Results: The fall in hemoglobin levels were between 2 and 6 g /dL, with an average of 3.1 g /dL between 7 and 10 post-operative days . A minimum hemoglobin value of 7.8 g/dL was noted between 7 and 10 days after surgery, while the mean reduction of serum iron was found to be 44.87 g/dL. Conclusion: The reduction in hemoglobin levels was responsible for the clinical symptoms. Iron supplementation was required in the immediate post-operative period.

La patologización del celibato en la medicina española (1820-1920) / The pathologization of celibacy in spanish medicine (1820-1920)

En la España de la Restauración, la novela naturalista y la prensa anticlerical insistían en que la causa principal de la depravación sexual de los sacerdotes era el voto de castidad. ¿En qué medida las tecnologías de saber y de poder médicos de la época permitían defender esa tesis? Este es el asunto abordado en el artículo. En primer lugar se examinan los antecedentes ilustrados de la ofensiva higienista contra el celibato sexual. En segundo lugar se analiza la controversia suscitada por Monlau con su defensa higiénica de la castidad sexual. Los argumentos de Monlau tienen lugar en un contexto de propaganda a favor del celibato suscitada por la Iglesia Católica. En tercer lugar se exploran los argumentos médicos que conectaban causalmente la continencia absoluta con las desviaciones sexuales, en particular la pederastia. Por último se indican las circunstancias que, a comienzos del siglo XX, llevaron a reactivar la defensa médica de la abstinencia sexual entre los jóvenes, anunciando un nuevo prototipo de masculinidad

In the Spain of the Restoration, the naturalist novel and the anticlerical press insisted that the main cause of the sexual depravity of priests was the vow of chastity. To what extent the technologies of knowledge and medical power of the time allowed to defend that thesis? This is the issue addressed in the article. First, we examine the enlightened background of the hygienist offensive against sexual celibacy. Secondly, the controversy raised by Monlau with his hygienic defense of sexual chastity is analyzed. The arguments of Monlau take place in a context of propaganda in favor of celibacy raised by the Spanish Catholic Church. Third, the medical arguments that causally connected absolute continence with sexual deviations, particularly "pederasty", are explored. Finally, we indicate the circumstances that, at the beginning of the 20th century, led to reactivate the medical defense of sexual abstinence among young people, announcing a new pattern of masculinity

Tuberculosis miliar con diseminación renal y genitourinaria: reporte de un caso / Miliary tuberculosis, with renal and genitourinary disemination; Case Report

Antecedentes: A pesar de los grandes avances en la medicina contemporánea; la Tuberculosis continúa siendo un reto diagnóstico, en especial al presentarse con características clínicas poco usuales. Caso clínico: Paciente masculino de 48 años, agri-cultor, de escasos recursos económicos, sin comorbilidades previas, el cual presenta sintomatología constitucional, pérdida de peso, iebre y dolor lumbar irradiado a ambos lancos de la región abdominal, de características poco especíicas, de un mes de evolución; acompañado de disuria y oliguria, y niega síntomas respiratorios. Al examen físico luce crónicamente enfermo, en mal estado nutricio-nal, sin deterioro de la conciencia, no se evidencia adenopatías cervicales ni inguinales; sin presencia de signos pulmonares, con leve dolor con la puño percusión renal bilateral, en el área genital, se identiica una masa de borde regular, móvil sobre el polo superior del testículo derecho. En el examen hematológico con presencia de Bicitopenia anemia microcitica hipocromica y trombocitopenia leve, en gases arteriales acidosis metabólica con Anión Gap elevado, además con hiperazoemia e hiperkalemia en la bioquímica sanguínea refractaria al tratamiento, razón por la cual fue sometido a Hemodiálisis aguda. Al evaluar radiografía de tórax se observa patrón micro-nodular difuso, no se realiza baciloscopias por falta de expectoración ni por lavado gástrico, en el ultrasonido renal y de vías urinarias se visualizó nefromegalia y la presencia de masa heterogénea de aspecto granulomatoso en testículo derecho; por lo que se solicitó tomografía toracoabdominal, en donde se observa patrón micronodular múltiple a nivel pulmonar, con afectación renal, ganglionar y testicular, se solicita BAAR de sedimento urinario seriado con resultados positivos, y la tinción Ziehl Neelsen en orina con resultado positivo; con ello se inicia terapia antiimica. Discusión: La Tuberculosis renal no tiene un cuadro clínico clásico; generalmente se presenta con manifestaciones atípicas; como en nuestro caso con sintomatología urinaria, hiperazoemia que culmino en terapia de restitución renal de emergencia, con posterior recuperación de la función renal.

Restless legs syndrome in pregnancy is connected with iron deficiency.

OBJECTIVE: Almost one-third of pregnant women develop symptoms of restless legs syndrome (RLS), which could have a negative impact on quality of life and the course of pregnancy and/or labor. The aim of our study was to determine possible risk factors for developing RLS in pregnant women. METHODS: A total of 300 random women in the third trimester of gravidity filled out a simple questionnaire based on the official diagnostic criteria for RLS. Respondents positive for RLS were interviewed to further characterize their symptoms. The parameters of iron metabolism were examined based on blood samples. All data were statistically analyzed. RESULTS: We detected significantly lower hemoglobin levels with signs of hypochromic anemia typical of iron deficiency in RLS-positive respondents, although oral iron supplementation was significantly higher in this group. The overall severity of symptoms correlated inversely with hemoglobin level. CONCLUSION: According to our results, it appears that serum iron level disturbances play a crucial role in the pathophysiology of secondary RLS in pregnant women.

Duodenal Ewing’s sarcoma: unusual location and atypical EWRS-1 translocation / Sarcoma de Ewing duodenal: localización inusual y traslocación atípica EWRS-1

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[Shock-induced anterior ischemic optic neuropathy from hemorrhagic anemia]. / Schockinduzierte anteriore ischämische Optikusneuropathie bei Blutungsanämie.

A 51-year-old diabetic and overweight male presented to our eye clinic with right-sided impairment of visual acuity and scotoma. A thorough work-up had been carried out at our institute 9 months prior to this event due to left-sided nonarteriitic anterior ischemic optic neuropathy (NAION). Despite the similarity of visual symptoms in the currently and previously affected eyes microcystic anemia (Hb 81 g/dl) associated with severe hemorrhoidal bleeding was diagnosed. The patient underwent surgical hemorrhoidectomy and received two red blood cell concentrates. Therefore, this article reports on an extraordinarily rare case of shock-induced anterior ischemic optic neuropathy (SIAION) caused by hemorrhoidal bleeding.

α-thalassemia-associated hydrops fetalis: A rare cause of thyrotoxic cardiomyopathy.

α°-thalassemia is a well-known cause of hydrops fetalis in South-East Asia and can be detected in utero. We report a very rare case of thyrotoxic cardiomyopathy associated with hyperplacentosis secondary to α°-thalassemia-associated hydrops fetalis. A 22-year-old primigravida with microcytic anemia presented at 27 weeks' gestation with pre-eclampsia, hyperthyroidism and cardiac failure. Serum ß-human chorionic gonadotrophin was markedly elevated and abdominal ultrasound revealed severe hydropic features and enlarged placenta. Serum ß-human chorionic gonadotrophin, cardiac function and thyroid function tests normalized after she delivered a macerated stillbirth. Histopathology of the placenta showed hyperplacentosis. Blood DNA analysis revealed that both patient and husband have the α°-thalassemia trait. This case illustrates a very atypical presentation of α°-thalassemia-associated hydrops fetalis and the importance of early prenatal diagnosis of α-thalassemia in women of relevant ethnic origin with microcytic anemia so that appropriate genetic counseling can be provided to reduce maternal morbidity and the incidence of hydrops fetalis.

Cerebral dural sinus thrombosis associated with adenomyosis: a case report.

We report a case of cerebral venous thrombosis (CVT) associated with a giant adenomyosis. At admission, the patient demonstrated generalized seizures and consciousness disturbance. Brain fluid-attenuated inversion recovery magnetic resonance imaging revealed a localized, high-intensity region in the left frontal lobe. Subsequent brain angiography showed that right internal carotid angiograms display abrupt termination of the anterior half of the superior sagittal sinus and a filling defect in the lateral part of the left transverse sinus. The patient complicated with iron deficiency anemia (IDA) and adenomyosis with higher levels of serum carbohydrate antigen 125 (CA125) and d-dimer. After 1 year from onset, intermittent severe menalgia and headache persisted, and blood examination revealed abnormal values; the patient was receiving oral medications. Finally, adenomyosis resection was performed with a favorable outcome, and no recurrence was observed during the 2-year follow-up period. We conclude that IDA and increased CA125 levels may have promoted hypercoagulability and CVT. This report emphasizes the possible relationship between CVT and adenomyosis.

[Safe dose rTPA for massive pulmonary embolism associated with high bleeding risk: a case report and review of the literature]. / Utilizzo di safe dose di rTPA nel trattamento dell'embolia polmonare massiva associata ad elevato rischio emorragico: caso clinico e revisione della letteratura.

Systemic thrombolysis is a well known treatment for massive pulmonary embolism (PE) but it remains often underutilized in clinical practice because of the risk of major bleeding, especially intracranial hemorrhage. Recently, the use of safe-dose recombinant tissue-type plasminogen activator (rTPA) has been proposed for the treatment of moderate PE demonstrating to be safe and more effective than standard anticoagulation. We report the case of an 83-year-old male patient affected by massive PE associated with high bleeding risk, and treated with half-dose of rTPA that resulted in rapid clinical improvement. This clinical experience led us to focus on the role of reduced doses of rTPA to decrease bleeding risk in patients with PE. We conclude that the new concept of "safe-dose thrombolysis" with rTPA may be considered a reasonable and interesting option in high-bleeding risk patients with massive PE.

Impact of major bleeding on long-term mortality in anemic versus nonanemic patients undergoing percutaneous coronary intervention using bivalirudin.

Bleeding complications after percutaneous coronary intervention (PCI) have been associated with higher short and long-term mortality. Bivalirudin has been shown to reduce bleeding complications in patients who underwent PCI; however, the impact of anemia on bleeding complications and long-term mortality has not been studied. A total of 11,991 patients who underwent PCI over a period of 8 years with bivalirudin as the primary antithrombotic agent were included. Anemia was defined according to the World Health Organization definition. Bleeding complications were prospectively collected. Survival analysis was performed using multivariable Cox proportional hazards models. Of the 11,991 patients, 4,815 patients (40%) had baseline anemia. Major bleeding occurred in 3.3% of patients with anemia compared with 0.7% of patients without anemia (p <0.001) driven largely by transfusion events. In the overall study population, major bleeding was a significant predictor of mortality (hazard ratio [HR] 1.4, 95% confidence interval [CI] 1.04 to 1.8, p = 0.027) at a mean follow-up of 2.6 years (interquartile range 1.4 to 3.5). In patients with anemia, major bleeding remained an independent predictor of mortality (HR 1.5, 95% CI 1.1 to 2.0, p = 0.008); however, in patients without anemia, it did not (HR 1.25, 95% CI 0.52 to 3.03, p = 0.62). In patients who underwent PCI with bivalirudin therapy, major bleeding is associated with early and long-term mortality, which is more pronounced in patients with baseline anemia.

Microcytic hypochromic anemia: should high performance liquid chromatography be used routinely for screening anemic and antenatal patients?

BACKGROUND: Hemoglobinopathies are the most common inherited red cell disorders worldwide. Identification of these disorders is immensely important epidemiologically and for improved management protocols. AIM AND OBJECTIVES: Our aim was to determine the prevalence of hemoglobinopathies in patients with microcytic hypochromic anemia and to assess the suitability of using high performance liquid chromatography (HPLC) routinely for screening antenatal cases and patients with anemia. MATERIALS AND METHODS: A total of 4335 cases received from Mar 2007 to Nov 2011 were studied for various hemoglobinopathies and variants on BIO RAD 'VARIANT' analyzer. RESULTS: Of the 4335 cases studied, 2119 were antenatal cases, 1710 patients with other disorders and 506 family studies. Of these, 688 cases displayed abnormal hemoglobin fractions on HPLC of which 140 were antenatal women. There were 455 cases of ß thalassemia trait, 24 ß thalassemia major, 20 thalassemia inter-media, 54 sickle cell trait, fivesickle cell disease, 21 double heterozygous ß thalassemia-sickle cell trait, nineand 4 Hb D- Punjab heterozygous and homozygous respectively, three Hb D ß Thalassemia trait, 20 and 37 Hb E homozygous and heterozygous respectively, three Hb E ß Thalassemia trait and four cases of Hb Q India. Twenty nine adults had isolated HbF elevation. CONCLUSION: Our study found a high prevalence (15.8%) of hemoglobinopathies amongst microcytic hypochromic anemia and antenatal cases. An accurate diagnosis helps in preventing unnecessary iron loading. Screening all antenatal cases with anemia helps in timely antenatal counseling, thus preventing the psychological trauma of bearing a transfusion dependent child for life.

Primary squamous cell carcinoma of the small intestine.

Squamous cell carcinoma of the small intestine is very rare. Only four cases have been reported so far. We report a case of primary squamous cell carcinoma arising in the small intestine and unusually causing strangulation of sigmoid colon in a 59-year-old female, the first case in India.

[Unusual aspect of pernicious anemia during association of beta-thalassemia: a new case report and literature review]. / Maladie De Biermer De Presentation Inhabituelle Sur Un Profil Beta Thalassemique : Apport D'Un Nouveau Cas Et Revue De La Litterature.

Pernicious anemia appears classically by macrocytosis. We report a case of a late discovered Biermer disease, on a 42-year-old young black woman. The reason was an unusual aspect of this disease in a context of betathalassemia. The patient presented chronic anemia which evolved during about ten year. Biology showed a normocytosis and signs of hemolysis according to beta-thalassemia. This was confirmed by an electrophoresis showing 9.1 % of fraction F some haemoglobin. Since this date, the patient was treated by folic acid alone with periodic transfusions of red blood cell. She presented eight years after the beginning of her disease, neurological deterioration. Diagnosis of pernicious anemia was finally established up on histological gastritis, low level of the blood rate of vitamin B12, macrocytosis, and presence of intrinsic anti-factor and parietal anti-cells antibodies.

Factors associated with and causes of perinatal mortality in northeastern Tanzania.

OBJECTIVE: To identify factors associated with perinatal mortality in northeastern Tanzania. DESIGN: Prospective cohort study. SETTING: Northeastern Tanzania. Population. 872 mothers and their newborns. METHODS: Pregnant women were screened for factors possibly associated with perinatal mortality, including preeclampsia, small-for-gestational age, preterm delivery, anemia, and health-seeking behavior. Fetal growth was monitored using ultrasound. Finally, the specific causes of the perinatal deaths were evaluated. MAIN OUTCOME MEASURE: Perinatal mortality. RESULTS: Forty-six deaths occurred. Key factors associated with perinatal mortality were preterm delivery (adjusted odds ratio (OR) 14.47, 95% confidence interval (CI) 3.23-64.86, p < 0.001), small-for-gestational age (adjusted OR 3.54, 95%CI 1.18-10.61, p = 0.02), and maternal anemia (adjusted OR 10.34, 95%CI 1.89-56.52, p = 0.007). Adherence to the antenatal care program (adjusted OR 0.027, 95%CI 0.003-0.26, p = 0.002) protected against perinatal mortality. The cause of death in 43% of cases was attributed to complications related to labor and specifically to intrapartum asphyxia (30%) and neonatal infection (13%). Among the remaining deaths, 27% (7/26) were attributed to preeclampsia and 23% (6/26) to small-for-gestational age. Of these, 54% (14/26) were preterm. CONCLUSIONS: Preeclampsia, small-for-gestational age and preterm delivery were key risk factors and causes of perinatal mortality in this area of Tanzania. Maternal anemia was also strongly associated with perinatal mortality. Furthermore, asphyxia accounted for a large proportion of the perinatal deaths. Interventions should target the prevention and handling of these conditions in order to reduce perinatal mortality.

α-Thalassemia mutations in two provinces of Southern Iran: Fars & Kohkeloye and Bouyer Ahmad.

The major defect in α-thalassemia (α-thal) is a single or double gene deletion. The aim of this study was to evaluate the α-thal mutations in two provinces of Southern Iran, Fars & Kohkeloye and Bouyer Ahmad. A total of 2000 subjects with microcytic hypochromic anemia was selected as a study group; finally, 1177 subjects were found to be α-thal mutation carriers by gap-polymerase chain reaction (gap-PCR) protocols and were confirmed by multiplex PCR and reverse hybridization test strips. A total of 1195 mutated α-globin alleles was identified in these patients that includes at least 10 different gene mutations. The most common mutation found was -α(3.7) (rightward deletion) (71.7%) and α(-5 nt) (IVS-I, -TGAGG) and the polyadenylation signal site (poly A1; AATAAA>AATAAG) were the second and third most common mutations (7.0 and 4.2%, respectively). In countries like Iran, with a high prevalence of thalassemia, identifying different mutations is helpful in carrier screening, genetic counseling and prenatal diagnosis of at-risk couples for the prevention of the birth of babies affected by severe thalassemic anomalies.

[A case of posterior reversible encephalopathy syndrome occurring after anemia correction].

A 53-year-old woman was admitted to our hospital with headache and convulsion. Advanced anemia with a Hb level of 3.5 g/dl had been detected about a month earlier, and it had been treated by iron administration to achieve a Hb level of 8.9 g/dl. The patient developed status epilepticus on admission. The blood pressure was elevated, and brain diffusion weighted imaging and fluid attenuated inversion recovery imaging revealed high intensity areas in the bilateral posterior and parietal lobes, right frontal lobe, and right basal ganglia. The cerebrospinal fluid protein was elevated. The convulsions settled after continuous infusion of thiamylal under mechanical ventilation. Subsequently, the patient became conscious, and the brain MRI abnormalities gradually disappeared. While a number of factors such as hypertension, medication and others have been reported as causes of posterior reversible encephalopathy syndrome (PRES), comparatively rapid anemia correction could also possibly precipitate PRES as like as this case. Thus anemia correction needs to be undertaken carefully.