1.How I Treat Primary Immune Deficiencies with Hematopoietic Stem Cell Transplantation
Hoon KOOK ; Boram KIM ; Hee Jo BAEK
Clinical Pediatric Hematology-Oncology 2022;29(2):35-43
Primary immune deficiencies (PID), or more recently, inborn errors of immunity (IEI), resulting from genetic defects of the immune system may present with increased susceptibility to infections, persistent inflammation, and autoimmunity. With recent introduction of next generation sequencing, the number of IEIs increases rapidly, reaching to 484 in 2022. Hematopoietic stem cell transplantation (HSCT) has been used over decades as a mainstay of specific treatment modality, while gene therapy and pharmacologic approach have been attempted with promising results in some PID in recent years. The survival following allogeneic HSCT for PID is now generally >80%. The indication and timing of transplant must be individualized not only on the basis of the specific PID but also on the characteristics of the individual patient. For the successful transplant outcome, the choice of donor and the optimal pretransplant conditioning regimen is important. This article will discuss current status and recommendations from specialists in HSCT for some representative PID, including severe combined immunodeficiency (SCID), CD40 ligand or CD40 deficiency, Wiskott-Aldrich syndrome, hemophagocytic lymphohistiocytosis, and chronic granulomatous disease, along with our personal experience of PID treatment in Korea.
2.Progress of Rehabilitation in Patients with Hypoglycemic Encephalopathy Accompanying Dysphagia and Voiding Difficulty: A Case Report.
Hannae JO ; Hee Won PARK ; Sora BAEK
Brain & Neurorehabilitation 2015;8(2):113-116
Dysphagia and voiding difficulty after hypoglycemic encephalopathy (HE) are not well described in the literature. Additionally, the effect of rehabilitation on outcomes of HE has not been discussed enough. Here we report two cases of HE, who underwent comprehensive rehabilitative management. A 76-year-old man with HE had cognitive dysfunction, dysphagia, poor standing balance, and voiding difficulty. After rehabilitation for about 20 days, the patient's swallowing, gait, and voiding function was improved remarkably, and he could eat a tolerable diet, walk independently, and void without catheterization. However, the cognitive function changed a little. A 75-year-old woman with HE had cognitive dysfunction, impaired gait, dysphagia, and voiding difficulty. After rehabilitation for one month, the patient made progress in swallowing and gait. However, the cognitive function changed a little. After rehabilitation, the recovery of swallowing and locomotor function was rapid and satisfactory in two cases, however, the progress of cognitive function was not definite.
Aged
;
Catheterization
;
Catheters
;
Deglutition
;
Deglutition Disorders*
;
Diet
;
Female
;
Gait
;
Humans
;
Rehabilitation*
3.Calcific Tendinopathy of the Gluteus Medius Mimicking Lumbar Radicular Pain Successfully Treated With Barbotage: A Case Report.
Hannae JO ; Gowun KIM ; Sora BAEK ; Hee Won PARK
Annals of Rehabilitation Medicine 2016;40(2):368-372
We report a case of calcific tendinopathy of the gluteus medius initially misdiagnosed as a lumbar herniated intervertebral disc. It was successfully treated with barbotage under ultrasonographic guidance finally. A 56-year-old woman was referred to interventional pain clinic for right hip pain due to an L5-S1 disc herniation. Serial L5 and S1 spinal nerve root blocks and epidural steroid injections were administered. However, pain relief was sustained only for a very short period. Plain radiography of the right hip revealed a solid calcific nodule at adjacent to the insertion site of the gluteus medius tendon. Physical modalities and extracorporeal shock wave therapy failed to improve the pain. Therefore, we attempted ultrasound-guided barbotage of the calcification. Barbotage was performed twice serially and her pain was considerably improved. At 6-month follow-up, the calcification was completely resolved.
Female
;
Follow-Up Studies
;
Hip
;
Humans
;
Intervertebral Disc
;
Middle Aged
;
Pain Clinics
;
Radiography
;
Shock
;
Spinal Nerve Roots
;
Tendinopathy*
;
Tendons
4.Study on CNS Oxytocinergic Pathway Projecting to the Mammary Nerve of the Rat.
Sang Ho BAEK ; Gee Dong KANG ; Eun Ah KIM ; Gyung Je JO ; Wan Sung CHOI ; Bong Hee LEE
Korean Journal of Anatomy 1997;30(6):705-712
To identify the central innervating the mammary nerve, viral retrograde transneuronal labelling methods were employed. Pseudorabies virus 6 microliter was injected into the mammary nerve of adult female Sprague-Dawley rats. After 4 days of survival, the animals were perfused with 4% paraformaldehyde-lysine periodate and their brains were processed for immunohistochemistry and double immunofluorescent staining of Pseudorabies virus or oxytocin using polyclonal antibodies. Several nuclei in brain were retrogradely labelled with Pesudorabies virus.Only a few magnocellular neurons of the paraventricular nucleus throughout whole brain showed double immunoreactivity to Pseudorabies virus and oxytocin. Approximately 11 percent of the oxytocinergic cells of the paraventricular nucleus,especially in three subnuclei[dorsomedial cap, lateral magnocelluar part and ventral part] showed double positive reaction to both Pseudorabies virus and oxytocin. These data demonstrate that some CNS cells projecting to the mammary nerve contained oxytocin and it may acts as a neurotransmitter in this pathway and a hormone targeting milk production and secretion.
Adult
;
Animals
;
Antibodies
;
Brain
;
Female
;
Herpesvirus 1, Suid
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Humans
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Immunohistochemistry
;
Milk
;
Neurons
;
Neurotransmitter Agents
;
Oxytocin
;
Paraventricular Hypothalamic Nucleus
;
Pseudorabies
;
Rats*
;
Rats, Sprague-Dawley
5.Long-term Neurological Complication of HHV-6 Encephalitis after Allogeneic Hematopoietic Stem Cell Transplantation
Bo Ram KIM ; Hee Jo BAEK ; Hoon KOOK
Clinical Pediatric Hematology-Oncology 2025;32(1):10-18
Background:
Human herpesvirus 6 (HHV-6) encephalitis is a rare but serious complication of allogeneic hematopoietic stem cell transplantation (HSCT). This study investigated the incidence, clinical features, and long-term neurological sequelae of HHV-6 encephalitis in pediatric and adolescent HSCT recipients.
Methods:
We retrospectively reviewed 92 patients who were younger than 20 years of age at the time of undergoing allogeneic HSCT between January 2015 and December 2024. HHV-6 encephalitis was diagnosed based on neurological symptoms and the detection of HHV-6 DNA in cerebrospinal fluid using multiplex polymerase chain reaction. Patients with HHV-6 encephalitis were followed for a median of six years (range, 4.5-8.5 years) to assess long-term neurological outcomes.
Results:
Three patients (3.2%) developed HHV-6 encephalitis between 15 and 26 days post-transplantation, coinciding with neutrophil engraftment. Clinical presentation included fever, seizures, altered consciousness, and short-term memory loss.Neuroimaging revealed high signal intensity lesions in the limbic system. Despite prompt antiviral therapy with foscarnet and initial viral clearance, all patients developed significant long-term neurological sequelae, including persistent cognitive impairment, epilepsy (two with refractory seizures), and memory disturbances. One patient continues to require assistance with activities of daily living, while two others face challenges reintegrating into school and society.
Conclusion
HHV-6 encephalitis following allogeneic HSCT in pediatric and adolescent patients can lead to severe and lasting neurological impairment, despite timely antiviral therapy. These long-term sequelae substantially affect quality of life and impose ongoing healthcare and societal burdens. Multidisciplinary long-term care is essential, and further research is warranted to improve prevention and treatment strategies.
6.Long-term Neurological Complication of HHV-6 Encephalitis after Allogeneic Hematopoietic Stem Cell Transplantation
Bo Ram KIM ; Hee Jo BAEK ; Hoon KOOK
Clinical Pediatric Hematology-Oncology 2025;32(1):10-18
Background:
Human herpesvirus 6 (HHV-6) encephalitis is a rare but serious complication of allogeneic hematopoietic stem cell transplantation (HSCT). This study investigated the incidence, clinical features, and long-term neurological sequelae of HHV-6 encephalitis in pediatric and adolescent HSCT recipients.
Methods:
We retrospectively reviewed 92 patients who were younger than 20 years of age at the time of undergoing allogeneic HSCT between January 2015 and December 2024. HHV-6 encephalitis was diagnosed based on neurological symptoms and the detection of HHV-6 DNA in cerebrospinal fluid using multiplex polymerase chain reaction. Patients with HHV-6 encephalitis were followed for a median of six years (range, 4.5-8.5 years) to assess long-term neurological outcomes.
Results:
Three patients (3.2%) developed HHV-6 encephalitis between 15 and 26 days post-transplantation, coinciding with neutrophil engraftment. Clinical presentation included fever, seizures, altered consciousness, and short-term memory loss.Neuroimaging revealed high signal intensity lesions in the limbic system. Despite prompt antiviral therapy with foscarnet and initial viral clearance, all patients developed significant long-term neurological sequelae, including persistent cognitive impairment, epilepsy (two with refractory seizures), and memory disturbances. One patient continues to require assistance with activities of daily living, while two others face challenges reintegrating into school and society.
Conclusion
HHV-6 encephalitis following allogeneic HSCT in pediatric and adolescent patients can lead to severe and lasting neurological impairment, despite timely antiviral therapy. These long-term sequelae substantially affect quality of life and impose ongoing healthcare and societal burdens. Multidisciplinary long-term care is essential, and further research is warranted to improve prevention and treatment strategies.
7.Long-term Neurological Complication of HHV-6 Encephalitis after Allogeneic Hematopoietic Stem Cell Transplantation
Bo Ram KIM ; Hee Jo BAEK ; Hoon KOOK
Clinical Pediatric Hematology-Oncology 2025;32(1):10-18
Background:
Human herpesvirus 6 (HHV-6) encephalitis is a rare but serious complication of allogeneic hematopoietic stem cell transplantation (HSCT). This study investigated the incidence, clinical features, and long-term neurological sequelae of HHV-6 encephalitis in pediatric and adolescent HSCT recipients.
Methods:
We retrospectively reviewed 92 patients who were younger than 20 years of age at the time of undergoing allogeneic HSCT between January 2015 and December 2024. HHV-6 encephalitis was diagnosed based on neurological symptoms and the detection of HHV-6 DNA in cerebrospinal fluid using multiplex polymerase chain reaction. Patients with HHV-6 encephalitis were followed for a median of six years (range, 4.5-8.5 years) to assess long-term neurological outcomes.
Results:
Three patients (3.2%) developed HHV-6 encephalitis between 15 and 26 days post-transplantation, coinciding with neutrophil engraftment. Clinical presentation included fever, seizures, altered consciousness, and short-term memory loss.Neuroimaging revealed high signal intensity lesions in the limbic system. Despite prompt antiviral therapy with foscarnet and initial viral clearance, all patients developed significant long-term neurological sequelae, including persistent cognitive impairment, epilepsy (two with refractory seizures), and memory disturbances. One patient continues to require assistance with activities of daily living, while two others face challenges reintegrating into school and society.
Conclusion
HHV-6 encephalitis following allogeneic HSCT in pediatric and adolescent patients can lead to severe and lasting neurological impairment, despite timely antiviral therapy. These long-term sequelae substantially affect quality of life and impose ongoing healthcare and societal burdens. Multidisciplinary long-term care is essential, and further research is warranted to improve prevention and treatment strategies.
8.Long-term Neurological Complication of HHV-6 Encephalitis after Allogeneic Hematopoietic Stem Cell Transplantation
Bo Ram KIM ; Hee Jo BAEK ; Hoon KOOK
Clinical Pediatric Hematology-Oncology 2025;32(1):10-18
Background:
Human herpesvirus 6 (HHV-6) encephalitis is a rare but serious complication of allogeneic hematopoietic stem cell transplantation (HSCT). This study investigated the incidence, clinical features, and long-term neurological sequelae of HHV-6 encephalitis in pediatric and adolescent HSCT recipients.
Methods:
We retrospectively reviewed 92 patients who were younger than 20 years of age at the time of undergoing allogeneic HSCT between January 2015 and December 2024. HHV-6 encephalitis was diagnosed based on neurological symptoms and the detection of HHV-6 DNA in cerebrospinal fluid using multiplex polymerase chain reaction. Patients with HHV-6 encephalitis were followed for a median of six years (range, 4.5-8.5 years) to assess long-term neurological outcomes.
Results:
Three patients (3.2%) developed HHV-6 encephalitis between 15 and 26 days post-transplantation, coinciding with neutrophil engraftment. Clinical presentation included fever, seizures, altered consciousness, and short-term memory loss.Neuroimaging revealed high signal intensity lesions in the limbic system. Despite prompt antiviral therapy with foscarnet and initial viral clearance, all patients developed significant long-term neurological sequelae, including persistent cognitive impairment, epilepsy (two with refractory seizures), and memory disturbances. One patient continues to require assistance with activities of daily living, while two others face challenges reintegrating into school and society.
Conclusion
HHV-6 encephalitis following allogeneic HSCT in pediatric and adolescent patients can lead to severe and lasting neurological impairment, despite timely antiviral therapy. These long-term sequelae substantially affect quality of life and impose ongoing healthcare and societal burdens. Multidisciplinary long-term care is essential, and further research is warranted to improve prevention and treatment strategies.
9.Corrigendum: Infection Control in Parenteral Nutrition Preparation and Compounding
Ji Hyeong CHOE ; Jin Hee BAEK ; Yun Hee JO ; Yoon Sook CHO
Journal of Clinical Nutrition 2019;11(1):29-30
The authors revised some errors regarding Table 5 and 6 in the article as the corrigendum. Because it is not official and may be changed in the revision due out in June 2019, Table 5 and 6 should be deleted and do not refer to it.
10.Infection Control in Parenteral Nutrition Preparation and Compounding.
Ji Hyeong CHOE ; Jin Hee BAEK ; Yun Hee JO ; Yoon Sook CHO
Journal of Clinical Nutrition 2018;10(2):31-37
Recently, in Korea, the importance of preparation and use of injectable drugs has been emphasized due to successive fatal accidents caused by injection infections. Parenteral nutrition (PN) has also been identified as a cause of infection. Cases of infection due to PN have been reported not only in Korea, but also abroad, and contamination occurs mainly during the preparation of PN. Because sterile preparation and compounding of injections are very important for infection control and patient safety, this article reviews the major guidelines outlined thus far. The Korea Ministry of Food and Drug Safety in 2006 published guidelines and the KSHP (Korean Society of Health-System Pharmacists) recently issued guidelines for the aseptic preparation of injections. In addition, as US guidelines, the ASHP (American Society of Health-System Pharmacists) guidelines and United States Pharmacopeia (USP) < 797 > are also reviewed. The recent guidelines published by the KSHP have significance in that they were adopted in accordance with the domestic reality, even though they conform to foreign guidelines, and are expected to be guidelines for hospital pharmacists performing aseptic preparation work. In addition, the Korea Ministry of Health and Welfare is considering appropriate guidelines for the safe management of medications, training staff for infection prevention and strengthening staff capacity. Furthermore, the gradual expansion of aseptic compounding facilities and human resources, as well as the provision of adequate medical costs are also considered. Based on the establishment and standardization of injectable drugs compounding guidelines for Korean hospitals, it is believed that if human resources and facilities are supported and medical charges are improved, it will be possible to expect the safer preparation and use of injections.
Drug Compounding
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Humans
;
Infection Control*
;
Korea
;
Parenteral Nutrition*
;
Patient Safety
;
Pharmacists
;
United States