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Importance: Iron-deficiency anemia (IDA) affects millions of persons worldwide, and is associated with impaired neurodevelopment in infants and children. Ferrous sulfate is the most commonly prescribed oral iron despite iron polysaccharide complex possibly being better tolerated.Objective: To compare the effect of ferrous sulfate with iron polysaccharide complex on hemoglobin concentration in infants and children with nutritional IDA.Design, Setting, and Participants: Double-blind, superiority randomized clinical trial of infants and children aged 9 to 48 months with nutritional IDA (assessed by history and laboratory criteria) that was conducted in an outpatient hematology clinic at a US tertiary care hospital from September 2013 through November 2015; 12-week follow-up ended in January 2016.Interventions: Three mg/kg of elemental iron once daily as either ferrous sulfate drops or iron polysaccharide complex drops for 12 weeks.Main Outcomes and Measures: Primary outcome was change in hemoglobin over 12 weeks. Secondary outcomes included complete resolution of IDA (defined as hemoglobin concentration >11 g/dL, mean corpuscular volume >70 fL, reticulocyte hemoglobin equivalent >25 pg, serum ferritin level >15 ng/mL, and total iron-binding capacity <425 μg/dL at the 12-week visit), changes in serum ferritin level and total iron-binding capacity, adverse effects.Results: Of 80 randomized infants and children (median age, 22 months; 55% male; 61% Hispanic white; 40 per group), 59 completed the trial (28 [70%] in ferrous sulfate group; 31 [78%] in iron polysaccharide complex group). From baseline to 12 weeks, mean hemoglobin increased from 7.9 to 11.9 g/dL (ferrous sulfate group) vs 7.7 to 11.1 g/dL (iron complex group), a greater difference of 1.0 g/dL (95% CI, 0.4 to 1.6 g/dL; P < .001) with ferrous sulfate (based on a linear mixed model). Proportion with a complete resolution of IDA was higher in the ferrous sulfate group (29% vs 6%; P = .04). Median serum ferritin level increased from 3.0 to 15.6 ng/mL (ferrous sulfate) vs 2.0 to 7.5 ng/mL (iron complex) over 12 weeks, a greater difference of 10.2 ng/mL (95% CI, 6.2 to 14.1 ng/mL; P < .001) with ferrous sulfate. Mean total iron-binding capacity decreased from 501 to 389 μg/dL (ferrous sulfate) vs 506 to 417 μg/dL (iron complex) (a greater difference of -50 μg/dL [95% CI, -86 to -14 μg/dL] with ferrous sulfate; P < .001). There were more reports of diarrhea in the iron complex group than in the ferrous sulfate group (58% vs 35%, respectively; P = .04).Conclusions and Relevance: Among infants and children aged 9 to 48 months with nutritional iron-deficiency anemia, ferrous sulfate compared with iron polysaccharide complex resulted in a greater increase in hemoglobin concentration at 12 weeks. Once daily, low-dose ferrous sulfate should be considered for children with nutritional iron-deficiency anemia.Trial Registration: clinicaltrials.gov Identifier: NCT01904864. [ABSTRACT FROM AUTHOR]

With the increase in electronic publications, and indeed the availability of existing publications in digital form, as well as the encouragement of open access publication, comes a challenge. That challenge is to create assistive software to aid in the discovery and selection of relevant documents to one's information need. Visual interfaces have begun to address the need of information seekers in finding publications and wading through the large result sets that are returned from search engines. There is currently, little evidence to suggest that these interfaces are based on systematic research on requirements. In this article, we examine areas which contribute to the theory and practice of visual interfaces directly relating to the discovery and selection of publications. We bring together work from different fields in a targeted approach to assist the future creation of these interfaces. [ABSTRACT FROM AUTHOR]

Importance: Sickle cell disease (SCD) is a life-threatening genetic disorder affecting nearly 100,000 individuals in the United States and is associated with many acute and chronic complications requiring immediate medical attention. Two disease-modifying therapies, hydroxyurea and long-term blood transfusions, are available but underused.Objective: To support and expand the number of health professionals able and willing to provide care for persons with SCD.Evidence Review: Databases of MEDLINE (including in-process and other nonindexed citations), EMBASE, Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Trials, CINAHL, TOXLINE, and Scopus were searched using prespecified search terms and keywords to identify randomized clinical trials, nonrandomized intervention studies, and observational studies. Literature searches of English-language publications from 1980 with updates through April 1, 2014, addressed key questions developed by the expert panel members and methodologists.Findings: Strong recommendations for preventive services include daily oral prophylactic penicillin up to the age of 5 years, annual transcranial Doppler examinations from the ages of 2 to 16 years in those with sickle cell anemia, and long-term transfusion therapy to prevent stroke in those children with abnormal transcranial Doppler velocity (≥200 cm/s). Strong recommendations addressing acute complications include rapid initiation of opioids for treatment of severe pain associated with a vasoocclusive crisis, and use of incentive spirometry in patients hospitalized for a vasoocclusive crisis. Strong recommendations for chronic complications include use of analgesics and physical therapy for treatment of avascular necrosis, and use of angiotensin-converting enzyme inhibitor therapy for microalbuminuria in adults with SCD. Strong recommendations for children and adults with proliferative sickle cell retinopathy include referral to expert specialists for consideration of laser photocoagulation and for echocardiography to evaluate signs of pulmonary hypertension. Hydroxyurea therapy is strongly recommended for adults with 3 or more severe vasoocclusive crises during any 12-month period, with SCD pain or chronic anemia interfering with daily activities, or with severe or recurrent episodes of acute chest syndrome. A recommendation of moderate strength suggests offering treatment with hydroxyurea without regard to the presence of symptoms for infants, children, and adolescents. In persons with sickle cell anemia, preoperative transfusion therapy to increase hemoglobin levels to 10 g/dL is strongly recommended with a moderate strength recommendation to maintain sickle hemoglobin levels of less than 30% prior to the next transfusion during long-term transfusion therapy. A strong recommendation to assess iron overload is accompanied by a moderate strength recommendation to begin iron chelation therapy when indicated.Conclusions and Relevance: Hydroxyurea and transfusion therapy are strongly recommended for many individuals with SCD. Many other recommendations are based on quality of evidence that is less than high due to the paucity of clinical trials regarding screening, management, and monitoring for individuals with SCD. [ABSTRACT FROM AUTHOR]

IMPORTANCE Sickle cell disease (SCD) is a life-threatening genetic disorder affecting nearly 100 000 individuals in the United States and is associated with many acute and chronic complications requiring immediate medical attention. Two disease-modifying therapies, hydroxyurea and long-term blood transfusions, are available but underused. OBJECTIVE To support and expand the number of health professionals able and willing to provide care for persons with SCD. EVIDENCE REVIEW Databases of MEDLINE (including in-process and other nonindexed citations), EMBASE, Cochrane Database of Systematic Reviews, Cochrane Central Register of Controlled Trials, CINAHL, TOXLINE, and Scopus were searched using prespecified search terms and keywords to identify randomized clinical trials, nonrandomized intervention studies, and observational studies. Literature searches of English-language publications from 1980 with updates through April 1,2014, addressed key questions developed by the expert panel members and methodologists. FINDINGS Strong recommendations for preventive services include daily oral prophylactic penicillin up to the age of 5 years, annual transcranial Doppler examinations from the ages of 2 to 16 years in those with sickle cell anemia, and long-term transfusion therapy to prevent stroke in those children with abnormal transcranial Doppler velocity (>200 cm/s). Strong recommendations addressing acute complications include rapid initiation of opioids for treatment of severe pain associated with a vasoocclusive crisis, and use of incentive spirometry in patients hospitalized for a vasoocclusive crisis. Strong recommendations for chronic complications include use of analgesics and physical therapy for treatment of avascular necrosis, and use of angiotensin-converting enzyme inhibitor therapy for microalbuminuria In adults with SCD. Strong recommendations for children and adults with proliferative sickle cell retinopathy include referral to expert specialists for consideration of laser photocoagulation and for echocardiography to evaluate signs of pulmonary hypertension. Hydroxyurea therapy is strongly recommended for adults with 3 or more severe vasoocclusive crises during any 12-month period, with SCD pain or chronic anemia interfering with daily activities, or with severe or recurrent episodes of acute chest syndrome. A recommendation of moderate strength suggests offering treatment with hydroxyurea without regard to the presence of symptoms for Infants, children, and adolescents. In persons with sickle cell anemia, preoperative transfusion therapy to increase hemoglobin levels to 10 g/dL is strongly recommended with a moderate strength recommendation to maintain sickle hemoglobin levels of less than 30% prior to the next transfusion during long-term transfusion therapy. A strong recommendation to assess iron overload is accompanied by a moderate strength recommendation to begin iron chelation therapy when indicated. CONCLUSIONS AND RELEVANCE Hydroxyurea and transfusion therapy are strongly recommended for many individuals with SCD. Many other recommendations are based on quality of evidence that is less than high due to the paucity of clinical trials regarding screening, management, and monitoring for individuals with SCD. [ABSTRACT FROM AUTHOR]

The governing equations for large deflections of cables have a highly nonlinear and coupled nature, which precludes exact analytical solutions except in a few simplified cases. The present study demonstrates the utility of Groebner Basis methodology in facilitating the construction of approximate analytical and semianalytical Galerkin solutions in the geometrically nonlinear analysis of cable statics. [ABSTRACT FROM AUTHOR]

Long-term follow-up of children with immune thrombocytopenia (ITP) indicates that the majority undergo remission and severe thrombocytopenia is infrequent. Details regarding bleeding manifestations, however, remain poorly categorized. We report here long-term data from the Intercontinental Cooperative ITP Study Group Registry II focusing on natural history, bleeding manifestations, and management. Data on 1345 subjects were collected at diagnosis and at 28 days, 6, 12, and 24 months thereafter. Median platelet counts were 214 × 109/L (interquartile range [IQR] 227, range 1-748), 211 × 109/L (IQR 192, range 1-594), and 215 × 109/L (IQR 198, range 1-598) at 6, 12, and 24 months, respectively, and a platelet count <20 × 109/L was uncommon (7%, 7%, and 4%, respectively). Remission occurred in 37% of patients between 28 days and 6 months, 16% between 6 and 12 months, and 24% between 12 and 24 months. There were no reports of intracranial hemorrhage, and the most common site of bleeding was skin. In patients with severe thrombocytopenia we observed a trend toward more drug treatment with increasing number of bleeding sites. Our data support that ITP is a benign condition for most affected children and that major hemorrhage, even with prolonged severe thrombocytopenia, is rare. [ABSTRACT FROM AUTHOR]

Abstract: Attentive reading is a complex and cognitively demanding task that uses note-taking and annotation to support the reader’s interpretation of the document. When reading on paper, extensive use of highlighting and other activities are conducted to support attentive reading, but this rich behaviour is not used with digital documents. Many users therefore print digital documents and then interact with them in physical form. This paper presents the “Digital Reading Desk,” an enhanced digital reading environment that provides support for attentive reading, providing a large working space for notes that mimics the use of desk space in a conventional physical environment. The Reading Desk uses a single tool to support both annotation and bookmarking, simplifying both the user’s learning of the system and their use of tools. Evaluation of the Reading Desk indicates preference for it, as well as considerable behavioural differences between our approach and traditional digital reading tools. [Copyright &y& Elsevier]

Romiplostim, a thrombopoietin-mimetic peptibody, increases and maintains platelet counts In adults with immune thrombocytopenia (ITP). In this first study of a thrombopoietic agent in children, patients with ITP of ≥ 6 months' duration were stratified by age 1:2:2 (12 months<3 years; 3-< 12 years; 12-< 18 years). Children received subcutaneous injections of romiplostim (n = 17) or placebo (n = 5) weekly for 12 weeks, with dose adjustments to maintain platelet counts between 50 × 109/L and 250 × 109/L. A platelet count ≥ 50 × 109/L for 2 con secutive weeks was achieved by 15/17 (88%) patients in the romiplostim group and no patients in the placebo group (P = .0008). Platelet counts ≥ 50 × 109/L were maintained for a median of 7 (range, 0-11) weeks in romiplostim patients and 0 (0-0) weeks in placebo patients (P= .0019). The median weekly dose of romipiostim at 12 weeks was 5 μg/kg. Fourteen responders received romiplostim for 4 additional weeks for assessment of pharmacokinetics. No patients discontinued the study. There were no treatment-related, serious adverse events. The most commonly reported adverse events in children, as in adults, were headache and epistaxis. In this short-term study, romiplostim increased platelet counts in 88% of children with ITP and was well-tolerated and apparently safe. The trial was registered with http://www.clinicaltrials.gov as NCT00515203. [ABSTRACT FROM AUTHOR]

Abstract: The governing strain-displacement and curvature-displacement equations for paraboloidal shells including shear deformation and rotary inertia are solved for free vibration of closed shells. The finite element method is used to obtain three-dimensional frequency of vibration solutions for a variety of boundary conditions, free, fixed and simply supported. Assumptions concerning the circumferential vibrational behavior are incorporated that reduce the analysis to a single coordinate and the element shape function is formulated using the meridional coordinate. The results for frequency of vibration compare favorably with the available literature. Selected results for frequency of vibration are presented in tabular form for several shell parameters, including free, pinned and fixed boundary conditions. Representative mode shapes are plotted for a fixed boundary condition. [Copyright &y& Elsevier]

Documents of all forms have always been at the centre of research in the humanities and arts. As more literature becomes available electronically, the impact of the digital medium on reading and research also grows. This article provides a review of key research on the limitations of digital reading, spanning a twenty-year period. Most of this literature is from the computer science field of human-computer interaction. From this backdrop, this article reports the main findings of a series of detailed studies that have investigated the current difficulties and limitations of digital document reader software. These studies, all conducted in the last four years, demonstrate that there are major limitations in digital document reader software that impede a number of simple interactions between user and document. It is also demonstrated that the current problems with this software can be overcome through a re-design of the human-computer interface. Ultimately, the current shortcomings of digital document reader software are a major impediment to realising the full potential of digital humanities. However, there is ample scope to make tangible improvements and progress is urgently needed. [ABSTRACT FROM AUTHOR]

The frequency of vibration of thick-walled toroidal shells is studied using a finite element formulation wherein the finite element is derived directly in toroidal coordinates. Hexagonal crystals of thallium and cadmium are used as representative transversely isotropic materials. The shell is assumed to be transversely isotropic with respect to the toroidal radial direction, and results based on that assumption are contrasted to a shell that is transversely isotropic with respect to the circumferential toroidal coordinate. It is established that an analysis based on a toroidal coordinate system is superior to an axisymmetric coordinate system and has some advantages over a commercial finite element code. Tables of results are presented that compare frequency of vibration for the above mentioned transversely isotropic materials and isotropic materials. [ABSTRACT FROM AUTHOR]

Abstract: A solution for the equations governing dynamic thermopiezoelectric behavior is assumed in the form of an exponential . The resulting formulation is reduced to one-dimension and will have both real and imaginary parts. The Galerkin method and assumed shape functions are used to illustrate the formulation of the resulting eigenvalue problem. [Copyright &y& Elsevier]

Almost all Web searches are carried out while the user is sitting at a conventional desktop computer connected to the Internet. Although online, handheld, mobile search offers new possibilities, the fast-paced, focused style of interaction may not be appropriate for all user search needs. The authors explore an alternative, relaxed style for Web searching that asynchronously combines an offline handheld computer and an online desktop personal computer. They discuss the role and utility of such an approach, present a tool to meet these user needs, and discuss its relation to other systems. [ABSTRACT FROM AUTHOR]

Abstract: A nine-node Lagrangian finite element is derived and implemented in elliptic coordinates. The element is used to formulate an analysis for the free vibration of an elliptical membrane. Frequencies and mode shapes are computed for vibration of both solid and annular elliptic membranes. The annular membrane is defined as a conformal ellipse. Both free and fixed interior boundary conditions are analyzed. Results for frequencies and mode shapes are presented in tabular and graphic format, respectively. [Copyright &y& Elsevier]

The article relates the author's experience of examining the City of David and the temple site at Zion in Jerusalem, Israel. It lists the questions about the city's situation that the author needs to be answered. It describes the ancient temples and fortresses built within the city. It also highlights the strategic locations of such temples and fortresses.

A finite element formulation in spherical coordinates is presented for the study of the vibrations of piezoelectric homogeneous and layered hollow spheres. The finite element model is based on nine-node Lagrangian interpolation functions. Representative cases are considered including solid elastic spheres and hollow homogeneous and laminated piezoelectric spheres. The accuracy of the proposed formulation is verified by comparison with existing solutions showing excellent agreement. Several new results are presented for different piezoelectric materials in both tabular and graphical format. [ABSTRACT FROM AUTHOR]

Several researchers have focused on developing material properties for homogeneous magneto-electro-elastic multiphase composite materials. The candidate materials for this study are piezoelectric BaTiO3 barium titanate as the embedded material with magnetostrictive CoFe2O4 cobalt iron oxide as the matrix material. The materials are evaluated in terms of modeling the physical problem of the free vibration an infinite plate. Multiphase material properties vary depending upon the ratio of fiber material to matrix material. Actual electromagnetic materials are modeled as layered materials with the ratio of constituent materials being controlled by varying the number and thickness of layers of each material. Frequencies of vibration are compared for the layered materials versus the multiphase materials as a measure of the accurateness of the derived material constants. Multiphase material predictions for frequency agree quite well with layered materials for the problem that is studied. [Copyright &y& Elsevier]

Discusses an evidence that support a claim regarding the source of water that flows through the Zion temple in Jerusalem. Accounts made by Yigael Yadin, editor of the Qumran Temple Scroll, about bathing place for temple priests; Information on the temple-fortress at Zion; Details of the water system in the area.

Presents an article on the discovery of the wall remains of the Tower of Siloam in Jerusalem. Contributions of archaeologists in the discovery of the wall; Objectives of building the tower and a temple; Reason behind the choice of location.

Small handheld devices—mobile phones, Pocket PCs etc.—are increasingly being used to access the web. Search engines are the most used web services and are an important factor of user support. Search engine providers have begun to offer their services on the small screen. This paper presents a detailed evaluation of the how easy to use such services are in these new contexts. An experiment was carried out to compare users'' abilities to complete search tasks using a mobile phone-sized, handheld computer-sized and conventional, desktop interface to the full Google™ index. With all three interfaces, when users succeed in completing a task, they do so quickly (within 2–3 min) and using few interactions with the search engine. When they fail, though, they fail badly. The paper examines the causes of failures in small screen searching and proposes guidelines for improving these interfaces. In addition, we present and discuss novel interaction schemes that put these guidelines into practice. [Copyright &y& Elsevier]

Focuses on a study which examined the vibration of thick isotropic spherical shells. Formulation of a nine-node Lagrangian finite element in spherical coordinates; Verification of the accuracy of the proposed analysis; Comparative study of frequencies of vibration for spherical shells for several definitions of a simple support; Analysis of results including free and fixed boundary conditions.

A nine-node Lagrangian finite element is formulated in an axisymmetric conical coordinate system and used to study the vibration of truncated hollow cones. The governing elasticity equations (strain-displacement) are derived using the generalized expressions for orthogonal curvilinear coordinates. The transformation for hexagonal material constants from a Cartesian coordinate system to the conical coordinate system is illustrated and results are presented for titanium and beryllium. Results for frequency and mode shape are presented in tabular form and graphically for hexagonal materials of class 6/mmm for a variety of truncated cone parameters. Vibration frequencies for isotropic cones are compared with similar results in the existing literature. [ABSTRACT FROM AUTHOR]

Abstract. Congenital red cell aplasia (Diamond-Blackfan anemia) is occasionally associated with hematologic defects other than a deficiency of red blood cell progenitors, but such alterations have not been well studied. The frequency and magnitude of abnormalities in platelet count and platelet function were therefore examined in 38 patients. Thrombocytosis was seen in 21 patients, and 12 had mild thrombocytopenia on at least one occasion. Elevated platelet counts were demonstrated repeatedly in nine children. The three patients with the lowest platelet counts also had leukopenia. Platelet aggregation was normal in all 16 patients in whom it was studied, and bleeding times were within the normal range in 14 of them. Bleeding signs and symptoms were not observed. It is concluded that thrombocytosis or thrombocytopenia often occurs but that platelet function is normal in patients with Diamond-Blackfan anemia. Pediatrics 68: 238-241, 1981; platelets, anemia, thrombocytosis. [ABSTRACT FROM AUTHOR]

Abstract. Of 49 patients with hemophilia A or B, who had not received replacement transfusions for at least 72 hours, ten were demonstrated to have a prolonged value for the bleeding time. None had evidence of yon Willebrand's disease or prior aspirin ingestion, and only one was found to have an associated intrinsic platelet defect. It appears that the bleeding time, generally thought to be a measurement of platelet-subendothelium interaction, may also sometimes be prolonged in congenital coagulation disorders in which platelet function is normal. Pediatrics 66:951-955, 1980; bleeding time, hemophilia. [ABSTRACT FROM AUTHOR]

ABSTRACT. Nine patients with severe classic hemophilia and inhibitors against factor VIII were treated for 156 bleeding episodes with 503 infusions of Proplex, Konyne, or Auto-Factor IX, three preparations of prothrombin complex concentrates (PCCs). Approximately two thirds of the bleeding episodes were managed successfully. Although the prothrombin time (PT) and partial thromboplastin time (PTT) were shortened after most PCC infusions, there was no evidence of disseminated intravascular coagulation. The degree of shortening of PT or PTT was not related to the particular PCC preparation used, dose, or cessation of hemorrhage. All PCC preparations contained activated clotting factors, as manifested by their ability to shorten the PTT of normal plasma, factor-VIII-deficient plasma, and factor-IX-deficient plasma. Shortening, which was greater with Auto-Factor IX than with the other products, was inhibited partially by a factor IX antibody and blocked completely by prolonged incubation with plasma. Although the nature of the procoagulant material in PCCs is uncertain, these products are of proven benefit to hemophilic patients with high-titer inhibitors. Side effects have been minimal and inhibitor titers have not risen. [ABSTRACT FROM AUTHOR]

Abstract. Selective erythroid aplasia occurred in two children receiving chemotherapy for acute lymphoblastic leukemia. The aplasia was reversible on cessation of therapy in one patient, and the other child remains transfusion-dependent on chemotherapy. Possible causes of pure red cell aplasia are discussed. Pediatrics, 59:895-898, 1977, ERYTHROID APLASIA, LYMPHOBLASTIC LEUKEMIA. [ABSTRACT FROM AUTHOR]

Abstract. Twenty-one children with chronic thrombocytopenia of obscure etiology, 19 of whom had normal numbers of bone marrow megakaryocytes, were evaluated in order to determine if factors extrinsic to the platelets themselves (presumably antibodies) caused the thrombocytopenia. The rapid destruction of platelets by such antibodies has been demonstrated to cause idiopathic thrombocytopenic purpura (ITP). History, physical examination, and routine laboratory studies were unable to provide an exact diagnosis in these patients. Measurement of platelet survival, using sodium chromate Cr 51 labeled homologous platelets obtained from unrelated donors, permitted a clear separation of these children into two groups. One group (11 patients) had a markedly shortened platelet life span, and was considered to have chronic ITP. The other group (10 patients) had a normal or only slightly reduced platelet life span. Thus, the destruction of platelets by extrinsic factors could be ruled out. Since the diagnosis of chronic ITP was excluded, splenectomy could be avoided in these patients since it is rarely useful in children with nonimmune forms of chronic thrombocytopenia. Homologous platelet survival measurement is a useful diagnostic tool in certain children with chronic thrombocytopenia. Pediatrics, 59:49-54, 1977, THROMBOCYTOPENIA, PLATELETS, PURPURA. [ABSTRACT FROM AUTHOR]

Provides information on neonatal bleeding. Medical approach to bleeding infant; Blood components used in therapy of bleeding infants; Hemorrhage secondary to platelet consumption in sick neonates; Information on bleeding in sick neonates with normal platelets.

Abstract. Mild leukopenia and thrombocytopenia are common in multitransfused hemophiliacs. Because little attention has previously been directed to measurements of erythropoiesis in these patients, we prospectively examined hemoglobin concentration and RBC indices in 94 children and young adults with hemophilia during comprehensive clinic visits. Additional studies performed in many included serum transferrin saturation, ferritin, haptoglobin, and free erythrocyte protoporphyrin measurements. Hemoglobin concentrations were recorded as age-related percentile values. Hemophiliacs of all ages and degrees of severity often had lower than average values for hemoglobin; 31% had values less than the third percentile, 46% less than the tenth percentile, and 83% less than the mean value. Reduced hemoglobin percentile values were unrelated to age, severity of disease, or human immunodeficiency virus antibody status. Only five patients had an obvious cause for anemia. Serum ferritin, transferrin saturation, and erythrocyte protoporphyrin values were usually normal, indicating that iron deficiency and anemia of chronic disease were uncommon. Although serum haptoglobin was reduced in 44% of the patients, reticulocyte count was infrequently increased. We conclude that hemoglobin values are frequently less than the mean normal values for age in hemophiliacs. Although frank anemia is common, it is usually mild and without obvious cause. Hemophiliac individuals with slightly reduced hemoglobin values probably do not routinely require detailed investigation for occult blood loss, iron deficiency, or inflammation. [ABSTRACT FROM AUTHOR]

Abstract. Although the epidemiology and pathophysiology of serious bacterial infection in homozygous sickle cell anemia (SS disease) have become increasingly well understood, information about infection risk and splenic reticuloendothelial function in hemoglobin SC disease is quite limited. Therefore, the type and frequency of invasive bacterial disease were examined in 51 children with SC disease followed for 370 person-years and splenic function was assessed in 31 patients by quantitation of pitted erythrocytes. Seven serious bacterial infections occurred in four of the patients, five due to Streptococcus pneumoniae and two to Haemophilus infiuenzae. A primary focus of infection was present in all episodes, none of which proved fatal. Although 30 episodes of pneumonia or chest syndrome occurred in 20 of the patients, a bacterial etiology was proven in only three instances. Splenic function was usually impaired, with a mean pit count of 7.1% +/- 8.2% (range 0% to 22.9%). This is significantly greater than normal, but less than pit counts in patients with SS disease or asplenic subjects. Children with SC disease may have a greater risk of bacterial infection than normal children, but their infection rate is not nearly as high as that in patients with SS disease. [ABSTRACT FROM AUTHOR]

Abstract. One measure used to prevent overwhelming sepsis due to Streptococcus pneumoniae in children with defective splenic function is oral penicillin prophylaxis. However, a frequently cited argument against this approach is the likelihood of poor compliance. Compliance was studied by examining urine specimens for penicillin by the Sarcina lutea disc diffusion technique in 22 surgically asplenic children, two patients following bone marrow transplantation, and 38 infants and young children with sickle cell disease. Multiple specimens (mean 3.5 per patient) were examined in 43 of the children. Overall, 125/188 (66%) of the urine samples contained penicillin, indicating compliance within the previous 12 to 24 hours. Compliance tended to improve on subsequent clinic visits. These relatively good results were attributed to an intensive educational program in which repetitive efforts are made to counsel patients and parents about the risks of life-threatening infection. Poor compliance should no longer be invoked as a reason not to study the efficacy of prophylactic penicillin in functionally or surgically asplenic subjects. Pediatrics 70:926-930, 1982; sickle cell, splenectomy, penicillin, Streptococcus pneumoniae, compliance. [ABSTRACT FROM AUTHOR]

Abstract. The clinical spectrum of hemolytic anemia as a consequence of oxidant stress in black children deficient in erythrocyte glucose-6-phosphate dehydrogenase (G-6-PD) has not been well described in the pediatric literature. During a 3 1/2-year period 14 hospitalized black G-6-PD-deficient children with moderate to severe hemolytic reactions were studied. The vast majority (13/14) were boys and were less than 3 years of age. Nine of the patients required blood transfusion. Eleven of the 14 episodes occurred with infection (five bacterial, six viral); those children with viral syndromes tended to have more severe hemolysis. Naphthalene was responsible for three episodes, but oxidant drugs were implicated in no instances. Findings on the blood smears of most subjects included irregular dense misshapen erythrocytes with asymmetrical distribution of hemoglobin and an adjacent membrane-bound clear zone ("eccentrocytes"). It is concluded that hemolytic reactions in the black G-6-PD-deficient child may be severe, are most commonly associated with infection, and are frequently characterized by distinctive erythrocyte morphology. [ABSTRACT FROM AUTHOR]

A letter from the authors of the article "Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members," which appeared in a 2014 issue.

The article presents comments of the author on a research by M.H. Steinberg and colleagues published in January 1, 2014 issue of the journal "Blood," which discusses the clinical importance of the distribution of fetal hemoglobin (HbF) within erythrocytes of persons with sickle cell anemia. It is noted that the author describes examples from the clinical sickle cell literature of diverse β globin haplotypes.

This section introduces a series of articles on the usability of digital libraries.

Focuses on a case of an infant with sickle cell anemia and acute splenic sequestration crisis. Characteristics of acute splenic sequestration crisis; Diagnosis; Treatment; Measures to reduce morbidity and mortality of infants due to acute splenic sequestration crisis.

Study Objective To assess the clinical severity and initial treatment of iron deficiency anemia (IDA) in female adolescents with heavy menstrual bleeding (HMB) in our center. Design Retrospective cohort study of electronic medical records via search of administrative records using International Classification of Diseases Ninth Revision codes for IDA or unspecified anemia and disorders of menstruation. Setting Children's Medical Center in Dallas, Texas. Participants One hundred seven patients with HMB and concomitant IDA (median age, 14.4 years) who presented to the outpatient, emergency department, and/or inpatient settings. Results The median initial hemoglobin concentration for all patients (n = 107) was 7.4 g/dL, and most (74%, n = 79) presented to the emergency department or via inpatient transfer. Symptomatic IDA was treated with blood transfusion in 46 (43%, n = 46). Ferrous sulfate was the most commonly prescribed oral iron therapy. Seven patients received intravenous iron therapy either initially or after oral iron treatment failure. Combined oral contraceptives were commonly prescribed for abnormal uterine bleeding, yet 10% of patients (n = 11) received no hormonal therapy during their initial management. Evaluation for underlying bleeding disorders was inconsistent. Conclusion Severe anemia because of IDA and HMB resulting in urgent medical care, including hospitalization and blood transfusion, is a common but underemphasized problem in adolescent girls. In addition to prevention and early diagnosis, meaningful efforts to improve initial management of adolescents with severe HMB and IDA are necessary. [ABSTRACT FROM AUTHOR]

Over 70,000 people live with sickle cell disease (SCD) in the United States and multitudes worldwide. About 2000 afflicted babies are born in this country each year. In African countries such as Nigeria, over 100,000 babies are born with the disease each year. Great strides have been made in the conservative management of SCD. However, the medical and psychosocial cost of supporting patients with this chronic illness is enormous and spans a lifetime. Hematopoietic stem cell transplantation (HSCT) can abrogate SCD manifestations, and is the best option for cure today. Yet, this treatment modality is underutilized as less than 500 transplants are reported in the Center for International Blood and Marrow Transplant Research (CIBMTR) database because of its significant risk of morbidity and mortality. There is growing understanding of the pathophysiology of the disease, and this, coupled with advances in transplantation and new approaches to therapy, continue to improve care of patients with SCD both in children and during adulthood. Continuing investigation seeks to predict the course of the disease and to determine timing and modality of therapy in order to optimize outcomes. [Copyright &y& Elsevier]