There are two pathogenic models of renovascular hypertension (RVH) originating from renal artery stenosis. We noted a case of a boy who had severe hypertension with atrophic left kidney, hypertrophic right kidney, a segmental stricture of the abdominal aorta, and total occlusion of the right renal artery. Due to the atrophic change of the contralateral, unclipped left kidney, this patient presented with various clinical manifestations related to both models of pathogenesis of RVH occurring at the same time. We conclude that this patient presented with the middle stage of the two RVH pathogenetic models, so called the 'one-clip, one and half-kidney model.'
Aorta, Abdominal ; Child* ; Constriction, Pathologic ; Humans ; Hypertension ; Hypertension, Renovascular* ; Kidney ; Male ; Renal Artery ; Renal Artery Obstruction ; Renin-Angiotensin System ; Takayasu Arteritis*

Purpose: Alport syndrome (AS) is one of the most common inherited renal diseases caused due to mutations of genes encoding specific proteins of the type IV collagen family, and its major clinical manifestations include progressive renal failure, sensorineural deafness, and ocular abnormalities. We investigated the clinical characteristics and long-term prognosis of AS in Korean pediatric and adult populations. Methods: We conducted a retrospective review of medical records of 33 children and adults who had been diagnosed or treated with AS from 1985 to 2019. Results: The mean age of the 33 patients diagnosed with AS was 16.2±13.6 years, and the male-to-female ratio was 2:1. At the first visit, recurrent gross hematuria was the most common initial symptom. In 10 of 33 patients (30.3%), sensorineural hearing loss (SNHL) was diagnosed, but none had ophthalmic problems. Moreover, 11 of 33 patients (33.3%) had advanced to end-stage renal disease (ESRD), and a significant difference was observed in the age of the patients who progressed to ESRD based on the presence or absence of SNHL (P =0.035). Conclusion SNHL in AS can be an important prognostic factor for long-term deterioration of renal function. Further investigation is required to confirm the clinical course and the genetic characteristics of AS in Korea through prospective national cohort studies.

In vivo electroporation has emerged as a leading technology for developing nonviral gene therapies, and the various technical parameters governing electroporation efficiency have been optimized by both theoretical and experimental analysis. However, most electroporation parameters focused on the electric conditions and the preferred vehicle for plasmid DNA injections has been normal saline. We hypothesized that salts in vehicle for plasmid DNA must affect the efficiency of DNA transfer because cations would alter ionic atmosphere, ionic strength, and conductivity of their medium. Here, we show that half saline (71 mM) is an optimal vehicle for in vivo electroporation of naked DNA in skeletal muscle. With various salt concentrations, two reporter genes, luciferase and beta-galactosidase were injected intramuscularly under our optimal electric condition (125 V/cm, 4 pulses x 2 times, 50 ms, 1 Hz). Exact salt concentrations of DNA vehicle were measured by the inductively coupled plasma-atomic emission spectrometer (ICP-AES) and the conductivity change in the tissue induced by the salt in the medium was measured by Low-Frequency (LF) Impedance Analyzer. Luciferase expression in-creased as cation concentration of vehicle dec-reased and this result can be visualized by X-Gal staining. However, at lower salt concentration, transfection efficiency was diminished because the hypoosmotic stress and electrical injury by low conductivity induced myofiber damage. At optimal salt concentration (71 mM), we observed a 3-fold average increase in luciferase expression in comparison with the normal saline condition (p < 0.01). These results provide a valuable experimental parameter for in vivo gene therapy mediated by electroporation.
Animals ; Comparative Study ; DNA/*administration & dosage/metabolism ; Drug Delivery Systems ; Electric Conductivity ; Electroporation/methods ; Escherichia coli/genetics ; Female ; Gene Therapy/*methods ; *Gene Transfer Techniques ; Genes, Reporter ; Injections, Intramuscular ; Luciferase/metabolism ; Mice ; Mice, Inbred BALB C ; Muscle, Skeletal/drug effects/*metabolism/pathology ; Osmolar Concentration ; Plasmids/genetics/*metabolism ; Sodium Chloride/*pharmacology ; Transfection ; Vehicles/*administration & dosage ; beta-Galactosidase/metabolism

In vivo electroporation has emerged as a leading technology for developing nonviral gene therapies, and the various technical parameters governing electroporation efficiency have been optimized by both theoretical and experimental analysis. However, most electroporation parameters focused on the electric conditions and the preferred vehicle for plasmid DNA injections has been normal saline. We hypothesized that salts in vehicle for plasmid DNA must affect the efficiency of DNA transfer because cations would alter ionic atmosphere, ionic strength, and conductivity of their medium. Here, we show that half saline (71 mM) is an optimal vehicle for in vivo electroporation of naked DNA in skeletal muscle. With various salt concentrations, two reporter genes, luciferase and beta-galactosidase were injected intramuscularly under our optimal electric condition (125 V/cm, 4 pulses x 2 times, 50 ms, 1 Hz). Exact salt concentrations of DNA vehicle were measured by the inductively coupled plasma-atomic emission spectrometer (ICP-AES) and the conductivity change in the tissue induced by the salt in the medium was measured by Low-Frequency (LF) Impedance Analyzer. Luciferase expression in-creased as cation concentration of vehicle dec-reased and this result can be visualized by X-Gal staining. However, at lower salt concentration, transfection efficiency was diminished because the hypoosmotic stress and electrical injury by low conductivity induced myofiber damage. At optimal salt concentration (71 mM), we observed a 3-fold average increase in luciferase expression in comparison with the normal saline condition (p < 0.01). These results provide a valuable experimental parameter for in vivo gene therapy mediated by electroporation.
Animals ; Comparative Study ; DNA/*administration & dosage/metabolism ; Drug Delivery Systems ; Electric Conductivity ; Electroporation/methods ; Escherichia coli/genetics ; Female ; Gene Therapy/*methods ; *Gene Transfer Techniques ; Genes, Reporter ; Injections, Intramuscular ; Luciferase/metabolism ; Mice ; Mice, Inbred BALB C ; Muscle, Skeletal/drug effects/*metabolism/pathology ; Osmolar Concentration ; Plasmids/genetics/*metabolism ; Sodium Chloride/*pharmacology ; Transfection ; Vehicles/*administration & dosage ; beta-Galactosidase/metabolism

There have been some cases where abnormal histopathologic findings could not be found in the kidney could even with proper specimen collection through percutaneous renal biopsy (PRB) in accordance with its indication. We analyzed the incidence and clinical outcomes of children who showed normal histopathological findings in their PRBs. Methods: The medical records of 552 pediatric subjects who underwent PRB between 2005 and 2016 were reviewed. Twenty-six subjects were excluded because allograft biopsy was performed in nine subjects, and the age at biopsy was greater than 18 years in 17 subjects. Finally, 526 subjects were enrolled in this study. Results: Of the 526 pediatric patients, 32 (6.1%) showed no histopathological abnormalities in their PRBs. The male-to-female ratio of the patients was 1.9:1, and the mean ages at the first visit and at biopsy were 10.6 ± 4.1 and 11.4 ± 3.8 years, respectively. In accordance with the biopsy indications, recurrent gross hematuria showed the highest incidence rate, but combined hematuria and proteinuria had the lowest incidence rate regarding normal renal histopathology among all the subjects. At a mean follow-up of 35.5 ± 23.6 months, urinary abnormalities had improved in more than 50% of the subjects with normal renal histopathology, and none of the patients showed progression to end-stage renal disease or required rebiopsy due to symptom worsening during the follow-up period. Conclusion: The clinical outcomes of children with normal PRB histopathologic findings are generally good. Further studies to evaluate their long-term outcomes are needed.

OBJECTIVE: To investigate the effects of functional magnetic stimulation (FMS) on the functional recovery in a rat model of spinal cord injury (SCI). METHOD: Forty-five Sprague-Dawley rats (200~250 grams, female) were used. Rats were laminectomized and the T9 segment of spinal cord was contused using New York University (NYU) spinal impactor. Ten gram weight rod was dropped from a height of 25 mm to produce moderately contused spinal cord injury model. The animals were randomly assigned to 2 groups: one exposed to FMS (FMS group) and the other not exposed to FMS (non-FMS group). Transcranial functional magnetic stimulation was noninvasively applied for 4 weeks. To compare the results between FMS group and non-FMS group, motor functions were evaluated with the Basso, Beattie, and Bresnahan (BBB) locomtor rating scale and inclined plane test, and somatosensory evoked potentials (SEP) and motor evoked potentials (MEP) were also recorded. RESULTS: There was a significant difference in locomotor recovery between FMS group and non-FMS group (p<0.05). Measurement of MEP was also indicated that amplitude of MEP in FMS group is larger than that in non-FMS group. CONCLUSION: These results indicate that FMS may have beneficial effects on motor recovery after spinal cord injury and the benefits of FMS could be an additional non-invasive therapeutic method for clinical trials in patients with spinal cord injury.
Animals ; Evoked Potentials, Motor ; Evoked Potentials, Somatosensory ; Humans ; Magnetics ; Magnets ; New York ; Rats ; Rats, Sprague-Dawley ; Spinal Cord ; Spinal Cord Injuries

BACKGROUND: Various systemic agents have been assessed for the treatment of alopecia areata (AA); however, there is a paucity of comparative studies. OBJECTIVE: To assess and compare cyclosporine and betamethasone minipulse therapy as treatments for AA with regard to effectiveness and safety. METHODS: Data were collected from 88 patients who received at least 3 months of oral cyclosporine (n=51) or betamethasone minipulse therapy (n=37) for AA. Patients with ≥50% of terminal hair regrowth in the alopecic area were considered responders. RESULTS: The responder of the cyclosporine group was 54.9% and that of the betamethasone minipulse group was 37.8%. In the cyclosporine group, patients with mild AA were found to respond better to the treatment. Based on the patient self-assessments, 70.6% of patients in the cyclosporine group and 43.2% of patients in the betamethasone minipulse group rated their hair regrowth as excellent or good. Side effects were less frequent in the cyclosporine group. CONCLUSION: Oral cyclosporine appeared to be superior to betamethasone minipulse therapy in terms of treatment effectiveness and safety.
Alopecia Areata* ; Alopecia* ; Betamethasone* ; Cyclosporine* ; Hair ; Humans ; Pulse Therapy, Drug ; Self-Assessment ; Treatment Outcome

Monoamniotic twin pregnancies are relatively rare, but perinatal mortality is high about 30-70%. The major cause of fetal death is cord accident, congenital anomalies, twin-twin transfusion syndrome, and intrauterine growth restriction. Especially cord entanglement occurs; perinatal mortality is about 50%. So the active management of monoamniotic twins to reduce the complication of cord entanglement is important. This is a case of monoamniotic twin pregnancy with cord entanglement and both fetuses survival by elective cesarean section at 32+4 weeks after medical amnioreduction and intensive fetal surveillance.
Cesarean Section ; Female ; Fetal Death ; Fetus ; Humans ; Perinatal Mortality ; Pregnancy ; Pregnancy, Twin

PURPOSE: Nutritional markers, such as total protein, albumin, and vitamin D have been reportedly associated with neonatal outcomes. This study aimed to assess the correlation between nutritional markers at birth and the need for respiratory support on the first day of life. METHODS: This retrospective study included 94 newborns admitted to the neonatal intensive care unit of Kyungpook National University Children's Hospital between March and December 2017. We measured levels of nutritional markers, including serum total protein, albumin, ferritin, 25-hydroxyvitamin D (25-OHD), and prealbumin, from cord blood or blood sample within 24 hours after birth. Respiratory support was defined as the use of nasal continuous positive airway pressure, humidified high-flow nasal cannula, or mechanical ventilation on the first day of life. RESULTS: The mean gestational age and birth weight were 36.6±2.3 weeks and 2,714±575 g, respectively. Serum total protein, albumin, prealbumin, and ferritin levels at birth were significantly correlated with gestational age and birth weight. Total protein, albumin, ferritin, and 25-OHD levels were not correlated with the time to recover birth weight after adjusting for gestational age. Moreover, prealbumin levels at birth were significantly lower in small-for-gestational-age infants than in appropriate-for-gestational-age infants. The need for respiratory support on the first day of life decreased 0.058- and 0.001-fold for every 1 g/dL increase in serum total protein (95% confidence interval [CI], 0.004 to 0.833; P=0.036) and albumin (95% CI, 0.000 to 0.136; P=0.009) levels, respectively. CONCLUSION: Nutritional status at birth could be associated with the need for respiratory support on the first day of life, regardless of the Apgar score.
Apgar Score ; Birth Weight ; Catheters ; Continuous Positive Airway Pressure ; Ferritins ; Fetal Blood ; Gestational Age ; Gyeongsangbuk-do ; Humans ; Infant ; Infant, Newborn ; Intensive Care, Neonatal ; Nutritional Status ; Parturition ; Prealbumin ; Respiration, Artificial ; Respiratory Insufficiency ; Retrospective Studies ; Vitamin D

OBJECTIVE: To investigate the pain-related behaviors and the changes of CX3CR1 expression in the dorsal root ganglion (DRG) in a rat model of lumbar disc herniation. METHOD: A total of 90 male Sprague-Dawley rats were used. A laminectomy was performed to expose left L5 nerve roots and corresponding DRG. Autologous nucleus puplosus was implanted on the left L5 nerve root proximal to the DRG without mechanical compression. Sham operation was also done with the same procedure as mentioned above. Thermal hyperalgesia and mechanical allodynia were assessed at 1, 5, 10, 20 and 30 days after surgery. Real time PCR and immunohistochemistry after behavioral test were performed. RESULTS: In the lumbar disc herniation rats, significant reduction of thermal withdrawal latency indicating thermal hyperalgesia was shown on the ipsilateral hindpaw on postoperative day 1 (p<0.01) and peaked on day 10 (p<0.05) and maintained throughout day 30 (p<0.05). The reduction of mechanical allodynia threshold, indicating mechanical allodynia, was observed on the ipsilateral hindpaw on postoperative day 1 (p<0.01) and continued throughout day 30 (p<0.01). Real time PCR showed the decrease in mRNA expression of CX3CR1 in the ipsilateral DRG on day 1 (p<0.05) and the significant increase on day 20 (p<0.05). The immunoreactivity for CX3CR1 was also increased in ipsilateral DRG on day 10 and 20. CONCLUSION: These data suggest that lumbar disc herniation induces thermal hyperalgesia and mechanical allodynia and upregulates the expression of CX3CR1 in dorsal root ganglion. Expression of CX3CR1 might be associated with subacute neuropathic pain after intervertebral disc herniation.
Animals ; Diagnosis-Related Groups ; Ganglia, Spinal ; Humans ; Hyperalgesia ; Immunohistochemistry ; Intervertebral Disc ; Laminectomy ; Male ; Neuralgia ; Rats ; Rats, Sprague-Dawley ; Real-Time Polymerase Chain Reaction ; RNA, Messenger ; Salicylamides ; Spinal Nerve Roots