Management of Renal Artery Aneurysms and an Arteriovenous Shunt in a Hemophilic Patient: A Case Report.

This case report details the management of a 66-year-old male with hemophilia A (HA) who presented with recurrent hematuria, and was found to have renal artery aneurysms and was subsequently diagnosed with a renal arteriovenous (AV) shunt. While the primary focus centers on the successful endovascular coil embolization of renal artery aneurysms, the concomitant presence of the AV shunt accentuates the significance of this case. Imaging techniques were crucial in the discovery of renal aneurysms and the diagnosis of the AV shunt malformation of the renal artery. This included an ultrasound, CT-angiography and digital subtraction angiography. The treatment approach employed prioritized endovascular coil embolization for its efficacy and reduced morbidity. Following the initial successful embolization, the identification of the AV shunt during subsequent embolization led to its targeted treatment. The case was also complicated by acute prostatitis that was treated medically. The patient's HA required careful administration of coagulation factor replacement therapy to control bleeding throughout the process. This case highlights the importance of reporting on the management of rare and complex pathologies to better understand and guide future treatments, especially involving this rare combination of renal AV shunts and hemophilia A.

Intradural dermoid cyst with complete dermal sinus of the posterior fossa: Contribution of 3D imaging with histopathological correlation.

A 5-year-old girl who presented with two episodes of meningitis, had a patchy red area and a small skin dimple in the midline of the occiput on physical examination. Imaging revealed a well-demarcated oval intradural lesion of the posterior fossa with restricted diffusion and peripheral enhancement, raising the possibility of an abscess. The 3D volume rendering of CT images of the inner surface of bone showed chronic bone remodeling and a tiny bone defect of the outer table. This detailed anatomical evaluation has an added value to MRI characteristics to orient for a preoperative diagnosis of an intradural dermoid cyst with a dermal sinus, that was confirmed by histopathological analysis after surgical excision.

Eltrombopag in pediatric chronic and refractory ITP: data from a retrospective multicenter study from Lebanon.

About 20% to 30% of pediatric patients with immune thrombocytopenia (ITP) develop chronic or refractory disease lasting 12 months or more that can be challenging to treat. Eltrombopag is being used after failure of previous lines of therapy with good results at tertiary healthcare centers in Lebanon, a developing country with available multidisciplinary treatment modalities. This is a retrospective multicenter observational study that analyzed data on pediatric patients with chronic or refractory ITP who were given eltrombopag as second- or third-line therapy in 6 large referral hospitals in Beirut (country capital located in mid Lebanon), South, North, and Mount Lebanon between October 2016 and May 2020. The primary end point of the study was a proportion of patients achieving platelet counts of ≥ 50 × 109/L for at least 6 weeks without requiring rescue therapy during the observation period. Data from 36 patients treated for chronic and refractory ITP, 20 (55.6%) males and 16 (44.4%) females, were analyzed. The median age at the eltrombopag dose was 11 years (2-18 years). All the patients had failure of the first-line therapy with steroids, 3 patients received eltrombopag as second-line therapy, and the remaining patients had failure of at least 2 previous lines of therapy, including steroids. The primary end point was achieved in 21 (58.3%) of 36 patients. The treatment was discontinued in 3 patients due to no response. Hepatotoxicity and all other adverse events (headache, weakness, and diarrhea) were mild and transient. All the patients who achieved the target platelet count of ≥ 50 × 109/L maintained the response for the treatment duration, which was a minimum of 5 months up to 3 years, with median/mean observation periods of 12 months and 14 months, respectively. This study confirms the findings of randomized controlled trials that eltrombopag as second- or third-line therapy in pediatric patients with chronic and refractory ITP is effective and safe, reinforcing its role in the real-world management of these patients.

Changing Management of Hematological Malignancies With COVID-19: Statement and Recommendations of the Lebanese Society of Hematology and Blood Transfusion.

COVID-19 caused by SARS-Cov-2 is a devastating infection in patients with hematological malignancies. In 2018, the Lebanese Society of Hematology and Blood Transfusion (LSHBT) updated the guidelines for the management of hematological malignancies in Lebanon. In 2019, it was followed by a second update. Given the rapidly changing evidence and general situation for COVID-19, the LSHBT established some recommendations and suggestions for the management of the patients with hematological malignancies taking into account the Lebanese condition, economic situation, and the facts that SARS-Cov-2 infection has apparently been devastating. In this article we present recommendations and proposals to reduce or to manage SARS-Cov-2 infection in the patients with myeloid and lymphoid hematological malignancies.

COVID-19 in benign hematology: emerging challenges and special considerations for healthcare professionals.

INTRODUCTION: Many patients with inherited or acquired benign hematological disorders are at increased risk of developing severe complications from COVID-19. These patients, therefore, require specific advice regarding isolation and changes to their usual treatment schedules. Their disease can also be associated with significant burden, and they necessitate life-long and regular access to therapy, and regular follow-up consultations and hospital visits. The current COVID-19 pandemic is therefore presenting many challenges for these patients, their families, and health-care professionals. AREAS COVERED: This review provides an overview of the reported COVID-19 cases in the literature in patients with certain benign hematological disorders including thalassemia, sickle cell disease, hemophilia, immune thrombocytopenia, venous thromboembolism, and aplastic anemia. The review also outlines some recommendations on how to manage these patients if they are infected with SARS-CoV-2. To review the literature on benign hematological disorders and COVID-19, a bibliographic search was performed using PubMed for articles published between January 2020 and June 2020. EXPERT OPINION: International efforts must be made to continue reporting and better understanding the effects of SARS-CoV-2 infection in these patients and accordingly develop a set of recommendations to optimize the treatment of future infected patients.

Impact of the coronavirus disease 2019 (COVID-19) pandemic on pediatric oncology care in the Middle East, North Africa, and West Asia region: A report from the Pediatric Oncology East and Mediterranean (POEM) group.

BACKGROUND: Childhood cancer is a highly curable disease when timely diagnosis and appropriate therapy are provided. A negative impact of the coronavirus disease 2019 (COVID-19) pandemic on access to care for children with cancer is likely but has not been evaluated. METHODS: A 34-item survey focusing on barriers to pediatric oncology management during the COVID-19 pandemic was distributed to heads of pediatric oncology units within the Pediatric Oncology East and Mediterranean (POEM) collaborative group, from the Middle East, North Africa, and West Asia. Responses were collected on April 11 through 22, 2020. Corresponding rates of proven COVID-19 cases and deaths were retrieved from the World Health Organization database. RESULTS: In total, 34 centers from 19 countries participated. Almost all centers applied guidelines to optimize resource utilization and safety, including delaying off-treatment visits, rotating and reducing staff, and implementing social distancing, hand hygiene measures, and personal protective equipment use. Essential treatments, including chemotherapy, surgery, and radiation therapy, were delayed in 29% to 44% of centers, and 24% of centers restricted acceptance of new patients. Clinical care delivery was reported as negatively affected in 28% of centers. Greater than 70% of centers reported shortages in blood products, and 47% to 62% reported interruptions in surgery and radiation as well as medication shortages. However, bed availability was affected in <30% of centers, reflecting the low rates of COVID-19 hospitalizations in the corresponding countries at the time of the survey. CONCLUSIONS: Mechanisms to approach childhood cancer treatment delivery during crises need to be re-evaluated, because treatment interruptions and delays are expected to affect patient outcomes in this otherwise largely curable disease.

Establishment of a formal program for retinoblastoma: Feasibility of clinical coordination across borders and impact on outcome.

Retinoblastoma is an ocular tumor that occurs in young children, in either heritable or sporadic manner. The relative rarity of retinoblastoma, and the need for expensive equipment, anesthesia, and pediatric ophthalmologic expertise, are barriers for effective treatment in developing countries. Also, with an average age-adjusted incidence of two to five cases per million children, patient number limits development of local expertise in countries with small populations. Lebanon is a small country with a population of approximately 4.5 million. In 2012, a comprehensive retinoblastoma program was formalized at the Children's Cancer Institute (CCI) at the American University of Beirut Medical Center, and resources were allocated for efficient interdisciplinary coordination to attract patients from neighboring countries such as Syria and Iraq, where such specialized therapy is also lacking. Through this program, care was coordinated across hospitals and borders such that patients would receive scheduled chemotherapy at their institution, and monthly retinal examinations and focal laser therapy at the CCI in Lebanon. Our results show the feasibility of successful collaboration across borders, with excellent patient and physician adherence to treatment plans. This was accompanied by an increase in patient referrals, which enables continued expertise development. However, the majority of patients presented with advanced intraocular disease, necessitating enucleation in 90% of eyes in unilateral cases, and more than 50% of eyes in bilateral cases. Future efforts need to focus on expanding the program that reaches to additional hospitals in both countries, and promoting early diagnosis, for further improvement of globe salvage rates.

Contribution of next generation sequencing in pediatric practice in Lebanon. A Study on 213 cases.

BACKGROUND: According to the Catalogue of Transmission Genetics in Arabs, less than half of diseases reported in Lebanese patients are mapped. In the recent years, Next Generation Sequencing (NGS) techniques have significantly improved clinical diagnosis, compared to traditional sequencing methods. METHODS: A total of 213 analyses by NGS (167 by whole exome sequencing (WES) and 46 by multigene panels tests) were performed on pediatric patients across different regions of Lebanon over a period of two years (December 2015-December 2017). RESULTS: Neurological disorders were the most frequent referral demand for both WES and gene panels (122/213). Pathogenic, likely pathogenic, or variants of unknown significance were identified in 69.5% of the WES and panel patients combined. Over half of the patients with such variants had an autosomal recessive disorder. A definite molecular diagnosis (pathogenic or likely pathogenic variants) was achieved in 34.1% and 47.8% of the patients studied by WES and the multigene panels, respectively. Thirty-three novel variants were found in the cases that were molecularly solved; 26 of these being identified by WES and seven by the multigene panels. In three consanguineous families, autosomal recessive inheritance of genes previously reported as showing dominant inheritance patterns were found. Biallelism was found in six cases, digenism in four cases, and one case was trigenic. CONCLUSION: Our study thus suggests that NGS tools are valuable for an improved clinical diagnosis, and highlights that the increased adoption of such techniques will significantly further improve our understanding of the genetic basis of inherited diseases in Lebanon.

Recurrent posterior reversible encephalopathy syndrome with cerebellar involvement leading to acute hydrocephalus.

Posterior reversible encephalopathy syndrome or PRES is a proposed cliniconeuroradiological entity that is characterized by headache, confusion, seizure, cortical visual disturbances or even blindness and, to a lesser extent, focal neurological signs. The etiology of this entity includes a sudden increase in blood pressure, renal failure, immunosuppressive drugs, infections, and intravenous immunoglobulin (IVIG). Classically, magnetic resonance imaging (MRI) findings show a symmetric reversible vasogenic edema in the parietooccipital lobes. PRES can involve the brainstem and cerebellum and sometimes can leave irreversible lesions but it can also recur, which is a very rare presentation. In this article, we report a case of recurrent PRES with cerebellar involvement associated with non-communicating hydrocephalus in a 2-year-old child with renal failure on peritoneal dialysis after receiving Etoposide for macrophage activation syndrome.

Collaborative Pediatric Bone Tumor Program to Improve Access to Specialized Care: An Initiative by the Lebanese Children's Oncology Group.

BACKGROUND: Children with malignant bone tumors have average 5-year survival rates of 60% to 70% with current multimodality therapy. Local control modalities aimed at preserving function greatly influence the quality of life of long-term survivors. In developing countries, the limited availability of multidisciplinary care and limited expertise in specialized surgery and pediatric radiation therapy, as well as financial cost, all form barriers to achieving optimal outcomes in this population. METHODS: We describe the establishment of a collaborative pediatric bone tumor program among a group of pediatric oncologists in Lebanon and Syria. This program provides access to specialized local control at a tertiary children's cancer center to pediatric patients with newly diagnosed bone tumors at participating sites. Central review of pathology, staging, and treatment planning is performed in a multidisciplinary tumor board setting. Patients receive chemotherapy at their respective centers on a unified treatment plan. Surgery and/or radiation therapy are performed centrally by specialized staff at the children's cancer center. Cost barriers were resolved through a program development initiative led by St Jude Children's Research Hospital. Once program feasibility was achieved, the Children's Cancer Center of Lebanon Foundation, via fundraising efforts, provided continuation of program-directed funding. RESULTS: Findings over a 3-year period showed the feasibility of this project, with timely local control and protocol adherence at eight collaborating centers. We report success in providing standard-of-care multidisciplinary therapy to this patient population with complex needs and financially challenging surgical procedures. CONCLUSION: This initiative can serve as a model, noting that facilitating access to specialized multidisciplinary care, resolution of financial barriers, and close administrative coordination all greatly contributed to the success of the program.

Observations on the Ministry of Public Health program of support to the hospitalization of patients in Lebanon.

Lebanon has a highly fragmented health care system. The Lebanese population receives its health care services through a system dominated by the private sector that is dependent to a large extent on public sector financing. Lebanon spends about 83% of its Gross Domestic Product (GDP) on health. This study consists of observations on the utilization of the Ministry of Public Health (MOH) program of hospital care provision. The study population included all patients admitted for hospitalization in any of the 126 hospitals contracted with the MOH, between August 2008 and July 2009 (one full year). This review is limited to medical admissions only. The surgical admissions have been excluded since they are covered under a 'flat fee' reimbursement. Findings reveal that a significant proportion of the hospital admissions under this program are for conditions that may not need hospitalization. Moreover, most of these admissions receive care in relatively small and peripheral hospitals. The findings ought to lead to a further scrutiny of the ministry program of support to the hospitalization of its nationals. Measures may be indicated to improve the efficiency and effectiveness of hospital utilization, avoid waste and possibly fraud, and reconsider the role of small and peripheral hospitals within the health care system of the country.

Pandemic (H1N1) 2009 influenza in pediatric hematology/oncology units in Lebanon.

BACKGROUND: The impact of pandemic (H1N1) 2009 influenza on immunocompromised patients in western countries has been described, but reports from pediatric patients in the Middle East or Arab countries are deficient. In this study, we describe the clinical characteristics of children with hematological malignancies and laboratory-proven H1N1 influenza. PATIENTS AND METHODS: Patients were recruited from 3 pediatric hematology/oncology units in Lebanon. A confirmed case of pandemic (H1N1) 2009 influenza is a clinically suspected case with positive H1N1 test by either a rapid immunofluorescence test or by real-time polymerase chain reaction. Data were collected retrospectively from the medical charts. RESULTS: From October 2009 to March 2010, 14 immunocompromised children were infected with H1N1 influenza. Eight were male and 6 were female. The median age of patients was 4.5 years (range, 1 to 14). All children were hospitalized and treated with oseltamivir. Twelve children responded to treatment; the other 2 patients with severe respiratory distress were transferred to intensive care unit and resuscitated but died after 7 and 12 days. CONCLUSIONS: Immunocompromised children infected with pandemic 2009 influenza may respond very well when the diagnosis and treatment are rapid. However, on the basis of our experience, if the underlying disease is more severe (immunodeficiency with significant immunosuppressive treatment and induction of high-risk leukemia), the odds of mortality are likely greater.

MECC regional initiative in pediatric palliative care: Middle Eastern course on pain management.

In all the major medical centers throughout the Middle East, there is a functioning pediatric hematology oncology department. In almost all countries, opioids such as morphine, oxycodone, and fentanyl are available. Pediatric palliative care services are still in their infancy and await further recognition and development. Unfortunately, there are still countries in the Middle East where children with cancer are diagnosed when the disease is already at stage III or IV, when the only option left is palliation. To decrease the incidence of late presentation, more effort is needed concerning public awareness, and concomitantly, an urgent need to develop hospital-based and community-based palliative and supportive care services. The initial step in this direction would involve more training of health care providers: Pediatricians, Pediatric Oncologists, Oncology Nurses, and Social Workers with updated pharmacological and nonpharmacological modalities of treatment.

Current status of pediatric hematology/oncology and palliative care in Lebanon: a physician's perspective.

Children with cancer suffer a lot, especially those in developing countries with limited health resources. Although pediatric hematology/oncology has become a well-developed subspecialty in Lebanon, pediatric palliative care is still underrecognized. The current pediatric hematology/oncology and palliative care services in Lebanon are reviewed in this article. We stress upon the adverse consequences of absence of well-defined pediatric palliative care in order for it to be recognized and integrated as a new discipline.

Limb salvage surgery for children and adolescents with malignant bone tumors in a developing country.

PURPOSE: To describe the 5-year experience of a multidisciplinary limb salvage program for children and adolescents with malignant bone tumors in Lebanon. PATIENTS AND METHODS: Between January 2002 to February 2007, 30 children and adolescents (2 with Ewing sarcoma and 28 with osteosarcoma) underwent Limb Salvage Surgeries (LSS) at the American University of Beirut Medical Center after partnering with the multidisciplinary team at St. Judes Children's Research Hospital (SJCRH). Procedures performed included 12 Repiphysis, noninvasively expandable, prostheses inserted in skeletally immature children, 15 modular prostheses, 2 allografts and 1 rotationplasty. All patients received pre- and postoperative chemotherapy. RESULTS: With a mean follow-up of 31 months, 20 patients are now off therapy, 4 died, and 6 are receiving chemotherapy (3 due to pulmonary recurrence). Complications of surgery included infections in three cases, failure of the expansion mechanism in two, femoral stem fracture in one, prostheses femoral stem loosening in one and wound dehiscence in one. Lengthening of Repiphysis prostheses was achieved by subjecting the limb to an electromagnetic field that would allow controlled release of the Repiphysis expansion mechanism. Ten patients underwent a total of 42 lengthening procedures with an average of 9 mm lengthened per procedure (range 2-15 mm). All patients have good function of the affected limb. CONCLUSION: Our LSS results are comparable to those reported in the literature. Collaboration with SJCRH and fund raising were critical to the program's success. This endeavor could serve as a model for establishing LSS programs in developing countries.

Molecular study of six families originating from the Middle-East and presenting with autosomal recessive osteopetrosis.

Autosomal recessive osteopetrosis is a severe hereditary bone disease whose cellular basis is in the osteoclast, but with heterogeneous molecular defects. We hereby report the clinical and the molecular study of seven patients affected by the recessive form of osteopetrosis (ARO) from six families originating from the Middle-East: four from Lebanon and two from Syria. Parental consanguinity was found in five families. The mean age of diagnosis was 3 months. Failure to thrive, prominent forehead, exophthalmia, optic atrophy, hepatosplenomegaly, neurological manifestations, anaemia, thrombocytopenia, hypocalcaemia, elevated hepatic enzymes and acid phosphatase, and an early fatal outcome were common. Macrocephaly, strabismus, and brain malformations were relatively less common. Mutations were identified in two genes: TCIRG1 and OSTM1. Phenotype-genotype correlation is discussed.