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1.
Pediatr Transplant ; 28(3): e14758, 2024 May.
Article in English | MEDLINE | ID: mdl-38659218

ABSTRACT

BACKGROUND: Data on the risk factors and outcomes for pediatric patients with SARS-CoV-2 infection (COVID-19) following hematopoietic stem cell transplantation (HSCT) are limited. OBJECTIVES: The study aimed to analyze the clinical signs, risk factors, and outcomes for ICU admission and mortality in a large pediatric cohort who underwent allogeneic HSCT prior to COVID-19 infection. METHOD: In this nationwide study, we retrospectively reviewed the data of 184 pediatric HSCT recipients who had COVID-19 between March 2020 and August 2022. RESULTS: The median time from HSCT to COVID-19 infection was 209.0 days (IQR, 111.7-340.8; range, 0-3845 days). The most common clinical manifestation was fever (58.7%). While most patients (78.8%) had asymptomatic/mild disease, the disease severity was moderate in 9.2% and severe and critical in 4.4% and 7.6%, respectively. The overall mortality was 10.9% (n: 20). Deaths were attributable to COVID-19 in nine (4.9%) patients. Multivariate analysis revealed that lower respiratory tract disease (LRTD) (OR, 23.20, p: .001) and lymphopenia at diagnosis (OR, 5.21, p: .006) were risk factors for ICU admission and that HSCT from a mismatched donor (OR, 54.04, p: .028), multisystem inflammatory syndrome in children (MIS-C) (OR, 31.07, p: .003), and LRTD (OR, 10.11, p: .035) were associated with a higher risk for COVID-19-related mortality. CONCLUSION: While COVID-19 is mostly asymptomatic or mild in pediatric transplant recipients, it can cause ICU admission in those with LRTD or lymphopenia at diagnosis and may be more fatal in those who are transplanted from a mismatched donor and those who develop MIS-C or LRTD.


Subject(s)
COVID-19 , Hematopoietic Stem Cell Transplantation , Humans , COVID-19/epidemiology , COVID-19/therapy , COVID-19/mortality , Hematopoietic Stem Cell Transplantation/adverse effects , Child , Male , Female , Retrospective Studies , Adolescent , Turkey/epidemiology , Child, Preschool , Risk Factors , SARS-CoV-2 , Infant , Transplantation, Homologous , Severity of Illness Index
2.
Pediatr Transplant ; 28(1): e14648, 2024 Feb.
Article in English | MEDLINE | ID: mdl-38063291

ABSTRACT

BACKGROUND: The aim of this study was to assess the impact of serum panel reactive antibodies (PRA) on the outcomes of allogeneic hematopoietic stem cell transplantation (HSCT) in pediatric thalassemia patients. METHODS: A total of 73 pediatric patients with thalassemia were included in this single-center study. Pre-transplant PRA levels were evaluated, and the patients were divided into two groups: PRA-negative (group 1; n = 44) and PRA-positive (group 2; n = 29). Patient characteristics, including age, gender, donor type, stem cell source, and HLA compatibility, were analyzed. Transplant outcomes, including engraftment, transfusion requirements, and transplant-related complications, were compared between the two groups. Further subgroup analysis was performed based on MFI values. RESULTS: At the time of transplantation, patients in group 1 were younger than those in group 2 (p = .008). The number of fully matched donors within the family (MSD and MFD) was significantly higher in group 1 (p = .049). Additionally, Rh blood group incompatibility was higher in group 2 (p = .03). There was no statistically significant difference in the engraftment days of neutrophils, platelets, and erythrocytes between the two groups. The frequency of poor graft function and graft failure was higher in the group 2, but there was no statistically significant difference. Post-transplant transfusion requirements for platelets and red blood cells were significantly higher in the group 2 (p < .001). Transplant-related complications such as VOD, PRES, and aGvHD were more common in the group 2, but no statistical significance was detected. CONCLUSIONS: Serum PRA in pediatric thalassemia patients may impact the outcomes of HSCT. PRA-positive patients had higher rates of blood product transfusion requirements. Although poor graft function, graft failure, and post-transplant complications were more common in the group 2, statistical significance was not observed. Identifying patients with high PRA levels can assist in optimizing transplant strategies and post-transplant care, leading to improved outcomes for the patients.


Subject(s)
Graft vs Host Disease , Hematopoietic Stem Cell Transplantation , Thalassemia , beta-Thalassemia , Humans , Child , Transplantation, Homologous , Hematopoietic Stem Cell Transplantation/adverse effects , Thalassemia/therapy , beta-Thalassemia/therapy , Tissue Donors , Retrospective Studies , Graft vs Host Disease/etiology
3.
Ups J Med Sci ; 115(2): 153-6, 2010 May.
Article in English | MEDLINE | ID: mdl-20095924

ABSTRACT

Peliosis is a rare benign disorder that is characterized by the presence of diffuse blood-filled cystic spaces and can occur in the liver, spleen, bone-marrow, and lungs. We present a 10-year-old boy with Fanconi anemia who presented with peliosis hepatis due to androgen treatment. Magnetic resonance (MR) imaging revealed multiple non-enhancing masses. Some of the lesions revealed fluid-fluid levels and extrahepatic extension on MR images. Diffusion-weighted (DW) imaging showed restricted diffusion. Fluid-fluid levels and extrahepatic extensions are unusual findings for hepatic peliotic lesions. In addition, DW imaging findings of peliosis hepatis have not been reported previously.


Subject(s)
Magnetic Resonance Imaging/methods , Peliosis Hepatis/diagnosis , Child , Fanconi Anemia/complications , Humans , Male , Peliosis Hepatis/complications , Peliosis Hepatis/pathology
4.
J Pediatr Hematol Oncol ; 31(10): 760-2, 2009 Oct.
Article in English | MEDLINE | ID: mdl-19755923

ABSTRACT

Immune hemolytic anemia is a rare condition in childhood. Cold agglutinins have been implicated in the etiology of the hemolysis and frequently observed during Mycoplasma pneumoniae infections. We present here a case of cold agglutinin-related hemolytic anemia, thrombocytopenia, and leukopenia secondary to M. pneumoniae associated pneumonia. It is suggested that even though very rare, M. pneumoniae infection should be considered as the underlying disease in a patient presenting with pancytopenia.


Subject(s)
Pancytopenia/etiology , Pneumonia, Mycoplasma/complications , Anemia, Hemolytic, Autoimmune/etiology , Child , Female , Humans , Leukopenia/etiology , Pneumonia, Mycoplasma/diagnosis , Thrombocytopenia/etiology
5.
J Pediatr Endocrinol Metab ; 22(2): 187-9, 2009 Feb.
Article in English | MEDLINE | ID: mdl-19449677

ABSTRACT

Congenital nephrogenic diabetes insipidus (CNDI) is a rare inherited disease characterized by renal tubular unresponsiveness to the antidiuretic effect of arginine-vasopressin due to the mutations of two molecules, the vasopressin V2 receptor (AVPR2) and the aquasporin-2 water channel. We report a novel AVPR2 mutation in a Turkish 18 month-old boy with skeletal anomalies.


Subject(s)
Diabetes Insipidus, Nephrogenic/genetics , Receptors, Vasopressin/genetics , Chromosomes, Human, X/genetics , DNA Mutational Analysis , Diabetes Insipidus, Nephrogenic/congenital , Diabetes Insipidus, Nephrogenic/diagnosis , Female , Genes, Recessive , Heterozygote , Humans , Infant , Male , Mothers , Pedigree , Radiography , Scoliosis/congenital , Scoliosis/diagnostic imaging , Scoliosis/genetics
6.
J Nephrol ; 19(2): 211-4, 2006.
Article in English | MEDLINE | ID: mdl-16736423

ABSTRACT

Rosai-Dorfman (R-D) disease is a benign lympho-histiocytosis of the lymphoid system. Immune derangement due to cytokine over-expression (tumor necrosis factor (TNF), interleukin (IL)-1b and IL-6) has been considered the cause of R-D disease. We present a 7-year-old boy with R-D disease who developed minimal change nephropathy (MCN) during the progression of R-D disease. The patient was resistant to oral prednisolone; and the remission of both R-D disease and MCN was achieved with oral cyclophosphamide (2 mg/kg, 12 weeks). MCN, the most common cause of nephrotic syndrome in childhood, is generally accepted to emerge by way of cytokine derangement. Correlation between R-D disease activity and the development and remission of nephrotic syndrome in our case suggested that nephrotic syndrome had been induced through some R-D disease-related immune mechanisms.


Subject(s)
Cyclophosphamide/administration & dosage , Histiocytosis, Sinus/drug therapy , Immunosuppressive Agents/administration & dosage , Nephrosis, Lipoid/drug therapy , Administration, Oral , Anti-Inflammatory Agents/administration & dosage , Child , Cytokines/immunology , Disease Progression , Drug Resistance/drug effects , Drug Resistance/immunology , Histiocytosis, Sinus/complications , Histiocytosis, Sinus/immunology , Histiocytosis, Sinus/pathology , Humans , Male , Nephrosis, Lipoid/etiology , Nephrosis, Lipoid/immunology , Nephrosis, Lipoid/pathology , Prednisolone/administration & dosage
7.
Pediatr Nephrol ; 21(5): 733-6, 2006 May.
Article in English | MEDLINE | ID: mdl-16491410

ABSTRACT

A 5-year-old girl with no underlying immune deficiency or hematologic disease was treated with a combination of ceftriaxone and ampicilline-sulbactam for pneumonia. On the ninth day of the therapy, she developed oliguria, paleness, malaise, immune hemolytic anemia (IHA) and acute renal failure (ARF). Laboratory studies showed the presence of antibodies against ceftriaxone. Acute interstitial nephritis (AIN) was diagnosed by renal biopsy. The patient's renal insufficiency was successfully treated with peritoneal dialysis without any complications. The patient recovered without any treatment using steroids or other immunosuppressive agents.


Subject(s)
Acute Kidney Injury/chemically induced , Anemia, Hemolytic/chemically induced , Anti-Bacterial Agents/adverse effects , Ceftriaxone/adverse effects , Nephritis, Interstitial/chemically induced , Acute Disease , Acute Kidney Injury/diagnosis , Acute Kidney Injury/immunology , Acute Kidney Injury/therapy , Ampicillin/adverse effects , Anemia, Hemolytic/diagnosis , Anemia, Hemolytic/immunology , Child, Preschool , Coombs Test , Female , Humans , Immunoglobulin G/immunology , Nephritis, Interstitial/diagnosis , Nephritis, Interstitial/immunology , Nephritis, Interstitial/therapy , Peritoneal Dialysis , Pneumonia/drug therapy , Sulbactam/adverse effects , Treatment Outcome
8.
J Clin Neurosci ; 13(1): 109-11, 2006 Jan.
Article in English | MEDLINE | ID: mdl-16410207

ABSTRACT

Pseudoephedrine is a nasal vasoconstrictor and is contained in many cough and cold medications. It is generally harmless when used in recommended doses. Hypertensive crisis, psychosis, hemorrhagic stroke, and intracranial hemorrhage have been reported as severe complications. We report a 4-year-old girl with nasopharyngeal rhabdomyosarcoma who developed stroke while on pseudoephedrine therapy for 3 days and had no hematological abnormalities.


Subject(s)
Ephedrine/adverse effects , Stroke/chemically induced , Child, Preschool , Female , Humans , Magnetic Resonance Imaging/methods , Rhabdomyosarcoma/drug therapy , Stroke/pathology , Vasoconstrictor Agents/adverse effects
10.
J Pediatr Hematol Oncol ; 27(1): 37-8, 2005 Jan.
Article in English | MEDLINE | ID: mdl-15654276

ABSTRACT

Ralstonia pickettii is an aerobic, gram-negative bacterium causing bacteremia following the use of contaminated saline vials, respiratory therapy solutions, skin disinfectants, blood culture mediums, and water supplies. It is rarely associated with human infections. The authors report two cases of R. pickettii bacteremia in patients with Port-A-Caths that could be treated only by removal of the ports.


Subject(s)
Bacteremia/etiology , Catheters, Indwelling/adverse effects , Catheters, Indwelling/microbiology , Gram-Negative Bacterial Infections/etiology , Oncology Service, Hospital , Anti-Bacterial Agents , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Bacteremia/drug therapy , Child, Preschool , Drug Therapy, Combination/therapeutic use , Equipment Contamination , Female , Humans , Infant , Infusions, Intravenous , Neoplasms, Germ Cell and Embryonal/drug therapy , Neoplasms, Neuroepithelial/drug therapy , Ralstonia
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