Bochdalek hernia presenting as recurrent fever and bicytopenia in a pediatric patient: A rare case report.

Bochdalek hernia is a rare condition characterized by the displacement of abdominal contents into the thoracic cavity. Due to the nonspecific nature of symptoms, prompt diagnosis, and management by emergency care providers can be challenging. Treatment of a Bochdalek hernia typically involves the reduction in the herniated contents back into the abdominal cavity. In this case report, we present the case of a 1-year-old girl who presented to the emergency department with a fever and bicytopenia. Further evaluation revealed a Bochdalek hernia, which was successfully managed with surgical intervention. This case highlights the importance of considering a Bochdalek hernia in the differential diagnosis of patients presenting with recurrent nonspecific symptoms (fever and bicytopenia).

Fanconi anemia phenotypic and transplant outcomes' associations in Iranian patients.

Objectives: Fanconi anemia (FA) is a rare, heterogeneous, inherited disorder. Allogeneic hematopoietic stem cell transplantation (HSCT) represents the only therapeutic option to restore normal hematopoiesis. This study reports the outcomes of FA-HSCT patients and identifies factors, including clinical phenotype. Our team examined more than 95% of Iranian FA patients during the last decade. Study Design: One hundred and six FA patients (age range: 2-41) who underwent HSCT from March 2007 to February 2018 were enrolled. Clinical characteristics of genetic disease, pre-HSCT findings, HSCT indication, and long-term follow-up evaluated and recorded. Data were analyzed using SPSS 19.0. Results: The mean follow-up period for survivors was 36 months (range, 1-101). The 3-year overall survival (OS) and disease-free survival were 72.2% and 71.2%, respectively. The 3-year OS rate for patients with limited and extensive malformations was 78.8% and 56.6%, respectively (p = 0.025). Acute graft versus host disease incidence was 60.52% for patients with limited malformations versus 70% for patients with extensive ones (p = 0.49). Chronic graft versus host disease incidence for these two groups was 9.21% and 10%, respectively (p = 0.91). Conclusions: OS was not associated with each of the malformations singly; however, it was lower in the extensive group. The younger age of patients at the HSCT time leads to a higher OS. The differences in FA patients' outcomes and the various genotypes were probably related. These data provide a powerful tool for further studies on genotype-phenotype association with HSCT results.

Total body irradiation-free haploidentical peripheral blood stem cell transplantation compared to related and unrelated donor transplantation in pediatrics with acute lymphoblastic leukemia.

BACKGROUND: Acute lymphoblastic leukemia (ALL) is the most prevalent childhood cancer under the age of 15 years. Despite the recent advances in therapeutic regimens, relapse occurs in 15%-20% of pediatric patients after chemotherapy, and hematopoietic stem cell transplantation (HSCT) is the best treatment option. However, donor availability is one of the major challenges. Over the last decade, haploidentical donor (HID) transplantation has evolved as an alternative option. Herein, we aimed to compare the transplant outcomes in pediatric patients receiving total body irradiation (TBI)-free myeloablative regimens, between non-HID and HID transplant. PATIENTS AND METHODS: The study included 60 pediatric ALL patients who had undergone HSCT from October 2016 until September 2020. Forty-three patients received non-HID HSCT, while 17 patients received HID. The sources of stem cells (SC) were peripheral blood stem cells (PBSC) for all the patients. The conditioning regimen was based on busulfan and cyclophosphamide. For graft-versus-host disease (GvHD) prophylaxis, patients received cyclosporine and methotrexate in the setting of non-HID transplantation, where HIDs received post-transplant cyclosporine and cyclophosphamide. RESULTS: The cumulative incidences of 3-year overall survival (OS) were 73.1%, 66.6%, and 69.5%, for matched sibling donor-matched related donor (MSD-MRD), matched unrelated donor-mismatched unrelated donor (MUD-MMUD), and HID groups, respectively (p = .85). The cumulative incidences of grade II-IV acute GvHD for the MRD, MUD-MMUD, and HID groups were 29%, 41%, and 49%, respectively (p = .47). Furthermore, the 3-year cumulative incidence of chronic GvHD was MSD-MRD: 70% versus MUD-MMUD: 42% versus HID: 45% (p = .64). The 3-year cumulative incidence of relapse post transplantation was 45%, 18%, and 45%, respectively, for the MSD-MRD, MUD-MMUD, and HID groups, and the differences were not statistically significant (p = .55). There was a higher risk for cytomegalovirus (CMV) infection in patients receiving HID transplants compared to those of non-HIDs (p < .01). CONCLUSION: Our results indicate that PBSC-HID transplant outcomes in the setting of non-TBI conditioning are comparable to those of non-HIDs in pediatric ALL patients.

Effect of Early Bacillus Calmette-Guerin Vaccination of Pediatric Severe Combined Immunodeficiency Patients on the Outcome of Hematopoietic Stem Cell Transplantation Using a Reduced-Intensity Conditioning Regimen.

The eminence of Bacillus Calmette-Guerin (BCG) vaccine in newborn vaccination programs has been conspicuous throughout the years, especially in low-income developing countries where tuberculosis is prevalent; however, application of the BCG vaccine is not without constraints, especially in patients afflicted with immunodeficiency diseases, such as severe combined immunodeficiency (SCID). The present study aimed to evaluate whether the administration of BCG vaccine at birth could improve the outcomes of hematopoietic stem cell transplantation (HSCT) in pediatric patients with SCID. In this study, 30 SCID patients who underwent HSCT using a reduced-intensity conditioning regimen (RIC) were followed-up for 2 years post-HSCT. The outcomes of HSCT were evaluated in both non-BCG-vaccinated patients (n = 12) and BCG-vaccinated patients (n = 18). Our results show a higher incidence of acute graft-versus-host disease (aGVHD), but not of chronic GVHD, in the BCG-vaccinated patients, and a similar overall survival (OS) rate in the 2 groups. We speculate that the similar OS rate in the 2 groups, despite the risk of BGC vaccination, was because this group received an RIC conditioning regimen. There was no other difference between the 2 groups. Considering the effect of the BCG vaccine on HSCT outcome, we suggest that the administration of BCG vaccine be deferred until age 3 months so that APT testing without the interference of maternal antibodies can be performed. However, this study could benefit from a larger cohort to further validate our findings, as the possible reason for some factors not being statistically significant was our small sample size.

The effect of suffering from rheumatoid arthritis, systemic lupus erythematosus, and back pain on sexual functioning and marital satisfaction in Iran.

OBJECTIVE: Sexual functioning is an important component of life quality and musculoskeletal disorders may effect sexual functioning, so, the present study was conducted to evaluate sexual functioning in patients suffering from back pain, rheumatoid arthritis, and systemic lupus erythematosus (SLE). METHODS: This study was conducted on 102 patients with rheumatoid arthritis, 103 patients with back pain, 103 patients with SLE, and 210 people in the control group by the consecutive sampling method. The marital satisfaction questionnaire (Enrich), Arizona Sexual Experience Scale (ASEX) questionnaire, and the General Health Questionnaire (GHQ-28) were completed by all the subjects. Disease severity was determined in each group of patients by Disease Activity Score of 28 joints, the Roland Morris questionnaire, and the SLE Disease Activity Index questionnaire. RESULTS: The GHQ in rheumatoid arthritis and lupus patients was meaningfully higher than the control group (P < .05), while there was no meaningful difference between back pain patients and the control group (P = .414). The sexual functioning questionnaire score in all 3 groups showed no statistically meaningful difference with the control group (P < .05). Also, the marital satisfaction questionnaire score in all the groups showed no statistically meaningful difference compared to the control group (P = .791). CONCLUSION: The study has shown that the level of sexual function in participants with back pain and the level of mental health and sexual functioning in patients with rheumatoid arthritis and SLE are significantly lower than healthy people and there is a need for intervention for improving mental health as well as sexual functioning in these patients.

High prevalence of latent tuberculosis in hematopoietic stem cell transplant recipients: A First Report.

TB is an increasing health problem, and patients undergoing HSCT are more prone to develop tuberculosis. The aim of our study was to evaluate prevalence of latent tuberculosis in HSCT recipients. In this study, 84 patients (2 months to 18 years) who were candidates for HSCT at the referral hospital of Tehran Children's Medical Center were enrolled. The TST and the QFT-GIT test were performed in all 84 patients, simultaneously. LTBI was considered when one of the tests was positive. Overall, the prevalence of LTBI in HSCT recipients in our study was 12% (10 cases). TST induration ≥5 mm was seen in only three patients (3.5%). Eight patients (9.5%) had a positive result for IGRA test, and 11 of them (13%) had indeterminate QFT-GIT result. The agreement between the TST results (induration size ≥5 mm) and the QFT-GIT results was poor (kappa = 0.14). In conclusion, there was a high rate of discordance between TST and IGRA results with many more positive QFT-GIT tests. However, more studies are needed in this population to determine whether this discordance reflects true infection.

Fasting Blood Glucose and Insulin Level in Opium Addict versus Non-Addict Individuals.

BACKGROUND: Many of lay person believe that opium lowers blood glucose. However some studies show the opposite results. In this study, we tried to evaluate the effect of opium on blood glucose and insulin resistance. METHODS: This comparative study including 53 addicts in case groups who used opium just in the form of smoking and 55 non-addicts in a control group, took part in the study, after proving not to be opium users. After taking blood samples, their fasting blood glucose (FBG), fasting blood insulin and lipid profiles were evaluated. Furthermore, insulin resistance index was analyzed via the homeostatic model assessment of insulin resistance (HOMA-IR) formula with the cut-off points of 7.2 and 7.1. FINDINGS: Age and gender were not significantly different between the groups. There was no significant difference regarding the prevalence of insulin resistance between the two groups, according to the cut-off points of 7.1 and 7.2 (P = 0.196 and P = 0.248, respectively). Mean insulin resistance index was not significantly different between the two groups (P = 0.325). In the case group, fasting blood insulin was considerably lower (P = 0.025) and fasting blood sugar (FBS) was significantly higher (P = 0.016) than the control group. CONCLUSION: According to the level of insulin and FBS in addicts, it does not seem that opium has a significant effect on reducing the blood glucose and insulin resistance.