Oncology Medical Training and Practice: Managing Jordan's Brain Drain Through Brain Train-The King Hussein Cancer Center Experience.

PURPOSE: The medical education system in Jordan is one of the most advanced education systems in the Middle East. Yet many medical school graduates leave the country to seek specialty and subspecialty education and training abroad, and the majority of graduates continue their careers there. METHODS: We explored reasons behind this so-called "brain drain" and how to slow it, along with capacity building opportunities and strategies for better local training. RESULTS: By taking advantage of various international collaborative opportunities, the King Hussein Cancer Center has managed to offer strong local training programs and an enhanced working environment, which has enabled us to improve the educational level of our graduates so they can help staff the Center, the country, and the region. CONCLUSION: Strong local training programs coupled with international partnerships can result in better training for physicians and offset the problem of brain drain without putting any restraints on the graduates.

Erratum to "Association of Tissue Transglutaminase Antibody Titer with Duodenal Histological Changes in Children with Celiac Disease".

[This corrects the article DOI: 10.1155/2016/6718590.].

Cancer care for adolescents and young adults in Jordan.

BACKGROUND: Adolescents and young adults with cancer have special characteristics and needs. AIMS: This study highlighted psychosocial challenges, fertility issues and secondary diseases encountered in adolescents and young adults with cancer. This work is meant to be a platform for future interventions for cancer in this demographic. METHODS: We investigated the latest edition of the Jordan Cancer Registry (JCR) and our more comprehensive institutional database during 2000-2012. Smoking, obesity and fertility preservation were addressed briefly as important issues among AYA patients. RESULTS: Cancer among adolescents and young adults represents 16.3% of all new cancer cases and has increased by 25% over the past 12 years. Women are more likely to be involved (female: male ratio of 1.44: 1) because of thyroid and breast cancers. Five-year survival rate for the AYA group was 72.4%, which was significantly better than for adults aged ≥ 40 years (59.8%) but worse than for paediatric patients aged < 15 years (79.2%) (P < 0.0001). CONCLUSIONS: Cancer in adolescents and young adults represents a substantial and growing proportion of oncological diagnoses. Due to their special needs and treatment complications, a dedicated service is urgently needed.

Review of management and morbidity of pediatric craniopharyngioma patients in a low-middle-income country: a 12-year experience.

BACKGROUND: Management of craniopharyngioma in children is challenging, and their quality of life can be significantly affected. Series describing this from low-middle income countries (LMIC) are few. PATIENTS AND METHODS: The study provides a retrospective chart review of pediatric patients <18 years old, diagnosed with craniopharyngioma between 2003 and 2014, and treated at King Hussein Cancer Center, Jordan. RESULTS: Twenty-four patients (12 males) were identified. Median age at diagnosis was 7.4 years (0.9-16.4 years). Commonest symptoms were visual impairment and headache (71%). Review of seventeen preoperative MRIs showed hypothalamic involvement in 88% and hydrocephalus in 76%. Thirteen patients (54%) had multiple surgical interventions. Five patients (21%) had initial gross total resection. Eleven patients (46%) received radiotherapy and six (25%) intra-cystic interferon. Five years' survival was 87 ± 7% with a median follow-up of 4.5 years (0.3-12.3 years). Four patients (17%) died; one after post-operative cerebral infarction and three secondary to hypothalamic damage. At their last evaluation, all but one patient required multiple hormonal supplements. Ten patients (42%) had best eye visual acuity (VA) >20/40, and four (16%) were legally blind. Eleven patients (46%) were overweight/obese; one had gastric bypass surgery. Seven patients had hyperlipidemia, and eight developed fatty liver infiltration. Eleven patients (65%) were attending schools and one at college. Nine of the living patients (53%) expressed difficulty to engage in the community. CONCLUSIONS: Management of pediatric craniopharyngioma is particularly complex and demanding in LMIC. Multidisciplinary care is integral to optimize the care and minimize the morbidities. A management outline for LMIC is proposed.

Association of Tissue Transglutaminase Antibody Titer with Duodenal Histological Changes in Children with Celiac Disease.

Celiac disease is usually diagnosed by demonstrating gluten enteropathy in small bowel biopsy. Celiac specific antibodies are used as an initial screening test. The goal of this study is to test the relationship of the anti-tTG titer and severity of histological changes in Jordanian children with celiac disease. Method. The medical records of 81 children who had elevated anti-tTG titer and had duodenal biopsies available were retrospectively reviewed. Result. Assessing the association of anti-tTG titer with duodenal histopathological changes, 94% of those with high anti-tTG titer (≥180 U/mL) had histological evidence of celiac disease. There was statistically significant positive association between high anti-tTG titer and Marsh grading as 82% of patients with Marsh III had high anti-tTG titer (Chi2 18.5; P value 0.00; Odds Ratio 8.5). The fraction of patients with Marsh III who were correctly identified as positive by anti-tTG titer ≥ 180 U/mL was high (sensitivity = 81.6). Moreover, the fraction of patients with anti-tTG titer ≥ 180 U/mL who had Marsh III was also high (positive predictive value = 78.4). Conclusion. Anti-tTG titer ≥ 180 U/mL had significant positive association with Marsh III histopathological changes of celiac disease.

Unrelated cord blood transplantation can restore hematologic and immunologic functions in patients with Chediak-Higashi syndrome.

CHS is a rare hereditary fatal disease, if not treated. APs occur in 85% of patients and are usually the main cause of mortality, and HSCT from HLA-matched related and unrelated donors is the only effective treatment for CHS and prevents recurrences of APs. We reviewed the records of three patients with CHS who underwent UCBT at KHCC. Records were examined for clinical features at the time of UCBT, conditioning regimens, morbidities, and outcomes. Conditioning comprised BU, cyclophosphamide, horse ATG, and etoposide. All patients tolerated the conditioning well. Two patients are alive, one with mixed and the other with full donor chimerism; hematologic and immunologic defects of CHS have been corrected in both patients. They show no evidence of recurrences of APs and have normal growth and development. In patients with CHS who lack HLA-matched related and unrelated donors, UCBT is a suitable alternative source of stem cells to restore immunologic and hematologic functions and prevent AP relapses, even in mixed chimeric states. Long follow-up and close monitoring are essential to evaluate the long-term benefits of using UCBT in patients with CHS.

Inflammatory myofibroblastic tumor causing unexplained anemia in a toddler: a case report.

INTRODUCTION: Inflammatory myofibroblastic tumor is a very rare benign tumor in children that mimics malignant tumors in its aggressiveness locally and by the possibility of recurrence after surgical resection, and causing anemia of chronic disease, which is a decrease in hemoglobin 1 to 2 g/dL below normal level in a patient with chronic illness. CASE PRESENTATION: A 32-month-old boy from Libya presented with microcytic hypochromic anemia. He had been treated in three countries and five centers without response to medical therapy. He was investigated at our center and found to have a mass in the colon causing intermittent intussusception and bleeding. He was treated surgically, and his condition improved dramatically. The pathology report proved a diagnosis of inflammatory myofibroblastic tumor. CONCLUSION: We report a case of an unusual tumor of the gastrointestinal tract causing chronic anemia not responding to medical treatment, and discuss the characteristics of inflammatory myofibroblastic tumor. In our case, we stress the involvement of a multidisciplinary team in treating such a patient who presents with common symptoms and signs but in whom there has been no response to any of the measures and treatment protocols.

Arthrogryposis, renal tubular acidosis and cholestasis (ARC) syndrome: two new cases and review.

ARC syndrome, the association of arthrogryposis, renal tubular dysfunction and cholestasis, is a rare genetic disorder. We report two Saudi infants from two different families with ARC syndrome. Magnetic resonance imaging of the brain of one of the infants showed lissencephaly, a previously unreported finding in this syndrome. We also review 39 ARC cases reported in the literature using the Medline database from January 1966 to September 2004.

Nephrocalcinosis in glucose-galactose malabsorption, association with renal tubular acidosis.

We report an association of renal tubular acidosis (RTA) in two children with glucose-galactose malabsorption (GGM), who were found to have nephrocalcinosis. Although GGM has been reported previously with nephrocalcinosis, this report is the first to show that renal tubular acidosis could explain the coexistence of nephrocalcinosis in patients with glucose galactose malabsorption.

Fatal lymphoproliferative disorder in a child with Schimke immuno-osseous dysplasia.

Schimke immuno-osseous dysplasia (SIOD) is a rare autosomal recessive disorder characterized by spondyloepiphyseal dysplasia, nephrotic syndrome, and cell-mediated immunodeficiency. Mutations in the SMARCAL1 gene (SW1/SNF2-related, matrix-associated, actin-dependent regulator of chromatin, subfamily a-like1) cause SIOD. We report a patient with SIOD and SMARCAL1 mutations, who presented with fever of unknown origin secondary to B-cell lymphoma. To our knowledge, this is the first report of an SIOD patient with a primary lymphoproliferative disorder (LPD).

Neonatal diabetes mellitus, congenital hypothyroidism, hepatic fibrosis, polycystic kidneys, and congenital glaucoma: a new autosomal recessive syndrome?

We report on two sibs (of 4) with a syndrome of minor facial anomalies, proportionate IUGR, neonatal non-autoimmune diabetes mellitus (NDM), severe congenital hypothyroidism (CH), cholestasis, congenital glaucoma, and polycystic kidneys. Liver disease progressed to hepatic fibrosis. The renal disease was characterized by large kidneys and multiple small cysts with deficient corticomedullary junction differentiation and normal kidney function. The phenotype observed in the two sibs was identical. Although a combination of liver, kidney, and pancreatic involvement has been described in Ivemark syndrome (hepato-renal-pancreatic syndrome), the coexistence of NDM, CH, and glaucoma in both sibs suggests the possibility that this combination of manifestations describes a new autosomal recessive syndrome. Mutation analysis for several candidate genes is warranted.