Calcineurin Inhibitor-Induced Type IV Renal Tubular Acidosis in Post-Bone Marrow Transplant: Review of Pathophysiology and Principles of Management.

Calcineurin inhibitors (CNI) are the mainstay of immunosuppressant medications in both bone marrow transplants and solid organ transplants. Nephrotoxicity is a well-known adverse effect of this group. Type IV renal tubular acidosis is a potentially under-recognized complication. Here we report a case of Omenn syndrome in a patient who underwent a bone marrow transplant and developed type IV renal tubular acidosis while on treatment with cyclosporine.

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.

Human Parvovirus B19 in Children with Sickle Cell Disease; Poking the Spleen.

Parvovirus is a known culprit of transient red cell aplasia (TRCA) in children with sickle cell disease (SCD). Few reports have previously described the association between the virus and acute splenic sequestration crisis (ASSC) in the same patient. Here, we are shedding light on such a potentially serious combination by reporting two cases of siblings with SCD complicated with concurrent ASSC and TRCA and presenting a review of the relevant literature.

Transfusion Consent in Oman: Physicians' Perception at a Tertiary Care University Hospital.

OBJECTIVES: Transfusion is a common intervention that mandates the discussion of benefits, risks, and alternatives to planned transfusions. In Oman, transfusion consent was first introduced at the Sultan Qaboos University Hospital in March 2014. We sought to evaluate our physicians' opinions, attitudes, and perception of the transfusion consent process. METHODS: Attending physicians of different specialties were invited to complete an anonymous survey on transfusion consent. RESULTS: A total of 114 physicians responded to the survey. Transfusion benefits and risks were explained regularly by 91% and 87% of the surveyed physicians, respectively. On the other hand, alternatives were declared by only 38%. Discomfort with the consent process was admitted by 10% of the physicians. There was no statistically significant association between discomfort in obtaining the consent and the physician seniority (p = 0.801), nor their specialties (p = 0.623). The importance of the consent process was acknowledged by 80% of surveyed physicians, who supported its implementation in other hospitals. CONCLUSION: This survey reflects positive attitudes of the surveyed physicians on the importance of transfusion consent. However, actions are required to achieve physicians' full ease with the process and to ensure that transfusion alternatives are discussed. We advocate implementation of transfusion consent in other hospitals in Oman.

Coexistence of sickle cell disease and severe congenital neutropenia: first impressions can be deceiving.

We report an Omani family in whom the propositus had a rare coexistence of sickle cell disease and severe congenital neutropenia associated with a mutation in ELANE. In contrast to his siblings with sickle cell disease, the severity of HbSS-associated complications such as painful crises and acute chest syndrome was significantly reduced. His course of the disease had markedly worsened after initiating G-CSF therapy. These clinical observations suggest that neutropenia may ameliorate inflammatory responses and thus display a modulating factor with respect to the clinical course of sickle cell disease.