Leishmaniasis is a vector-borne disease caused by protozoan parasites of the genus Leishmania. It is transmitted by phlebotomine female sand flies of the genera Phlebotomus and Lutzomyia in the old and new world, respectively. More than 20 well-recognized Leishmania species are known to infect humans and cause visceral (VL), cutaneous (CL) and mucocutaneous (ML) forms of the disease. Approximately 350 million people are at risk of contracting the disease and an estimated 1.6 million new cases occur annually. The disease mainly affects poor people in Africa, Asia and Latin America, and is associated with malnutrition, population migration, poor residency conditions, frail immune system and lack of resources. Previously, diagnosis of leishmaniasis relied mainly on invasive techniques of detecting parasites in splenic and bone marrow aspirates. Nevertheless, serological tests using the recombinant kinesin antigen (rK39) and molecular methods (polymerase chain reaction) are considered the best options for diagnosis today, despite problems related to varying sensitivities and specificities and field adaptability. Therapy of leishmaniasis ranges from local treatment of cutaneous lesions to systemic often toxic, therapy for disseminated CL, ML and VL. Agents with efficacy against leishmaniasis include amphotericin B, pentavalent antimonial drugs, paromomycin and miltefosine. No single therapy of VL currently offers satisfactory efficacy along with safety. This article provides a brief and updated systematic review on the epidemiology, diagnosis and treatment of this neglected disease.

/pdf/leishmaniasis-revisited-current-aspects-on-epidemiology-33sk5xnfw3.pdf

Leishmaniasis revisited: Current aspects on epidemiology, diagnosis and treatment

Compassionate use of bevacizumab (Avastin®) in children and young adults with refractory or recurrent solid tumors

Alpha thalassemia disorders are a group of hereditary anemias caused by absent or decreased production of the alpha chain of hemoglobin. Hemoglobin Bart’s hydrops fetalis is usually a fatal in-utero disease caused by absence of the alpha genes. However, the molecular and genotypic expression of hemoglobin Bart’s varies and increasing numbers of births are being reported. Population screening and prenatal diagnosis of at-risk couples is essential but often not performed. Most affected pregnancies are often undetected, resulting in severe fetal and maternal complications. Noninvasive monitoring by Doppler ultrasonagraphy with intrauterine transfusion therapy has changed the prognosis for this disorder. These advances in intrauterine and postnatal therapy have resulted in ethical dilemmas for the family and the provider.

/pdf/alpha-thalassemia-major-new-mutations-intrauterine-1jhj6znv2g.pdf

Alpha thalassemia major—new mutations, intrauterine management, and outcomes

a b s t r a c t In high-income countries the public policy consensus is that costs of delivering high-quality equitable cancer care present an increasing challenge to national budgets. In the U.S. alone it is estimated cancer care expenditures in 2020 will be 157 billion dollars. The increase is being driven by a number of factors including technological innovation, rising costs of medical and hospital care, expensive therapeutics and an increase in the proportion of individuals susceptible to malignancy as the population ages. In this article we review what factors are informing and influencing the political debate on cancer economics across Europe and North America. We have undertaken a comprehensive analysis of the literature and supplemented this with key informant interviews within each region. An important theme is the increasing role of individual patients, organisations and physicians in advocating for greater access to and fairer prices for cancer therapies. Whilst health technology assessments (HTAs) are increasingly prevalent their role in inform- ing reimbursement policy is influenced by public and political scrutiny, which impacts their ability to ensure access to high value cost effective care. Austerity measures following the global recession have created inequities in access to drugs with concern about the impact on subsequent outcomes. The cancer economics debate has largely centred on the provision of drugs, with access to radiotherapy and over-penetration of high cost radiation technologies under-represented in media outputs and political

Cancer economics, policy and politics: What informs the debate? Perspectives from the EU, Canada and US

An international registry of survivors with Hb Bart's hydrops fetalis syndrome.

An unusual form of bezoar extending from the stomach to the small intestine or beyond has been described as Rapunzel syndrome Bezoars are concretions in the gastrointestinal tract that increase in size by continuous accumulation of non-absorbable food or fibers Most bezoars in children are trichobezoars from swallowed hair from the head, dolls, or brushes Trichobezoars typically cause abdominal pain and nausea, but can also present as an asymptomatic abdominal mass, progressing to abdominal obstruction and perforation Trichobezoar with Rapunzel syndrome is an uncommon diagnosis in children with less than 40 cases reported It is predominantly found in emotionally disturbed or mentally retarded youngsters We present the youngest case of Rapunzel syndrome in the United States, a 5-year-old girl with mental retardation who presented with abdominal pain, vomiting and a non-tender abdominal mass

/pdf/rapunzel-syndrome-a-comprehensive-review-of-an-unusual-case-p4ek0h9u3s.pdf

Rapunzel syndrome: a comprehensive review of an unusual case of trichobezoar.

Hematologic causes of hydrops fetalis include homozygous α-thalassemia and immune hemolytic anemias. We report the case of a boy with hydrops fetalis who had homozygous α-thalassemia and alloimmune hemolytic anemia due to anti-E and anti-C blood group antibodies. He received intrauterine red blood cell transfusions and postnatal chronic transfusion with iron chelation therapy. A non-myeloablative sibling stem cell transplant failed. He is now 5 years and 6 months of age, hypothyroid with short stature, but in overall good health. He is one of the oldest reported homozygous α-thalassemia survivors and, to our knowledge, the only survivor with immune- and nonimmune-induced hydrops fetalis.

/pdf/hydrops-fetalis-caused-by-homozygous-alpha-thalassemia-and-4ohwog5vut.pdf

Hydrops fetalis caused by homozygous alpha-thalassemia and Rh antigen alloimmunization: report of a survivor and literature review.

Renal cell carcinoma is a chemo- and radiation-resistant malignancy that is very rare in childhood. In advanced cases, survival is extremely poor, but cure can be achieved with complete tumorectomy. We report a 14-year-old female with left renal cell carcinoma, with regional lymphadenopathy extending up to the diaphragm. Complete tumor resection was impossible. The tumor progressed after 10 months of treatment with high-dose interleukin-2. After 7 months of outpatient vascular endothelial growth factor receptor antibody bevacizumab and no significant adverse effects, dramatic tumor shrinkage enabled complete resection. Pediatr Blood Cancer 2008;50:903–904. © 2007 Wiley-Liss, Inc.

Vascular endothelial growth factor (VEGF) receptor antibody bevacizumab (avastin) induces regression of renal cell carcinoma in an adolescent resulting in residual tumorectomy

To the Editor: Dexamethasone is important in the management of acute lymphoblastic leukemia (ALL) and is used in nearly all phases of treatment. A review of children receiving corticosteroids for asthma or allergies showed that 40% developed restlessness and irritability and 60% depressed mood. In adults, these disorders are described as having a subacute onset, manic predominance and sometimes psychotic features [1]. The symptoms seem to be independent of age and steroid dose and occur more frequently in females [2]. They can be debilitating. Often, out of guilt and selfdoubt, parents do not seek help [3]. Corticosteroids are believed to decrease serotonin levels particularly in the hippocampus [4], and this may be related to abnormal psychological responses. We were able to completely resolve one child’s steroid-induced mood disorder with the use of a selective serotonin-reuptake inhibitor (SSRI). A 4-year-old female with ALL on standard-risk maintenance chemotherapy had been experiencing irritability with outbursts so severe that she was unable to attend kindergarten. She was receiving dexamethasone, 3 mg/m/dose orally twice a day for 5 days every month. The situation resulted in social isolation from family members, friends, peers and an inability to engage in ageappropriate activities, resulting in significant tension in the family. The child appeared to suffer from her inability to control this behavior. Psychological counseling did not improve symptoms. We used Citalopram at a very low dose (2.5 mg orally—0.1 mg/ kg/dose once daily). Most pediatric references quote effective ranges from 5 to 20 mg daily, with incremental increases of 5 mg/ week. The parents noted significant improvement in her ‘‘mean’’ and ‘‘angry’’ behaviors in 2 weeks, with complete resolution 2 weeks later. She now is only mildly moody when fatigued. Citalopram was chosen based on the experience of the institution’s psychiatrist with this agent in young children. While the debate on the effect of SSRI’s increasing the incidence of suicide in depressed children continues [5], this agent was found to have a favorable drug interaction profile compared to other SSRI’s, and was well tolerated in a depressed pediatric oncology population [6]. We administer Citalopram continuously, not just during steroid bursts. She has had no side effects. No steroid dose was modified or omitted. No changes were made to chemotherapy, and there was no clinical evidence of Citalopram interfering with chemotherapy. Distressing psychological problems from steroids during chemotherapy need to be proactively addressed and may be pharmacologically resolved without interrupting corticosteroids. Further study of these psychoactive agents should be pursued.

Treating dexamethasone-induced mood disorders in children with leukemia.

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Papers

Rapunzel syndrome: a comprehensive review of an unusual case of trichobezoar.

Hydrops fetalis caused by homozygous alpha-thalassemia and Rh antigen alloimmunization: report of a survivor and literature review.

Vascular endothelial growth factor (VEGF) receptor antibody bevacizumab (avastin) induces regression of renal cell carcinoma in an adolescent resulting in residual tumorectomy

Treating dexamethasone-induced mood disorders in children with leukemia.