Renal Syndromic Hearing Loss Is Common in Childhood-onset Chronic Kidney Disease

Background@#Hearing loss (HL) in children may adversely affect their development. HL is more prevalent in patients with chronic kidney disease (CKD) than in the general population.This study evaluated the prevalence of HL and its underlying diseases in patients with childhood-onset in CKD. @*Methods@#In this retrospective study of a tertiary referral center, childhood-onset CKD patients (stage 2–5, age at onset of renal symptom < 18 years) were recruited. We referred to the “renal” syndromic HL as cases with genetic or syndromic diseases, or extra-renal anomalies in addition to HL and CKD. @*Results@#A total of 421 patients (male:female = 279:142) were reviewed according to the causes of CKD: congenital anomalies of the kidney and urinary tract (CAKUT; n = 184, 43.7%), glomerulopathies (GP; n = 105, 24.9%), cystic kidney diseases (CYST; n = 39, 9.3%), perinatal problems (PP; n = 29, 6.9%), and others (n = 64, 15.2%). HL was detected in 82 (19.5%) patients, including 51 (12.1%) patients with sensorineural hearing loss (SNHL), 30 (7.1%) with conductive hearing loss (CHL), and 1 patient with mixed HL. The prevalence of HL in each group was as follows: 16.8% in the CAKUT group, 28.6% in the GP group, 12.8% in the CYST group, 24.1% in the PP group, and 14.1% in the others group. HL was more common in higher CKD stages, especially CHL in end-stage renal disease. SNHL was more prevalent in CKD from GP. Of the 82 patients with HL, 50% had renal syndromic HL: 58.8% of SNHL and one-third of CHL were renal syndromic HL. @*Conclusion@#One-fifth of the childhood-onset CKD had HL. Collectively, renal syndromic HL comprised half of the HL in this study. To improve the quality of life in patients with childhood-onset CKD, we suggest that HL should be considered, requiring surveillance, and if necessary, early intervention.

Quercetin Directly Interacts with Vitamin D Receptor (VDR): Structural Implication of VDR Activation by Quercetin

The vitamin D receptor (VDR) is a member of the nuclear receptor (NR) superfamily. The VDR binds to active vitamin D3 metabolites, which stimulates downstream transduction signaling involved in various physiological activities such as calcium homeostasis, bone mineralization, and cell differentiation. Quercetin is a widely distributed flavonoid in nature that is known to enhance transactivation of VDR target genes. However, the detailed molecular mechanism underlying VDR activation by quercetin is not well understood. We first demonstrated the interaction between quercetin and the VDR at the molecular level by using fluorescence quenching and saturation transfer difference (STD) NMR experiments. The dissociation constant (K(d)) of quercetin and the VDR was 21.15 ± 4.31 µM, and the mapping of quercetin subsites for VDR binding was performed using STD-NMR. The binding mode of quercetin was investigated by a docking study combined with molecular dynamics (MD) simulation. Quercetin might serve as a scaffold for the development of VDR modulators with selective biological activities.

Trochanteric Stress Fracture in a Female Window Cleaner / 대한고관절학회지

Stress fractures may occur at various sites in the femur including the head, neck, shaft, supracondylar and condylar regions. To the best of our knowledge, stress fracture occurring in the trochanteric region has not been previously reported. We report here a case of trochanteric stress fracture in a 53-year-old female window cleaner treated with hip nailing without adverse consequences. Careful consideration of this entity is needed when evaluating patients who have repetitive jumping up and down.

Radiological Assessment of the Effect of Congenital C3-4 Synostosis on Adjacent Segments

STUDY DESIGN: Retrospective case series. PURPOSE: To assess the effect of non-kyphotic aligned congenital C3-4 synostosis on the adjacent segment in 10 patients. OVERVIEW OF LITERATURE: In the cervical spine, fusion disease at the adjacent motion segments may be a risk factor for potential neurological compromise and death. METHODS: Radiograms of 10 patients 13 to 69 years of age presenting with neck/shoulder discomfort or pain with or without trauma history were examined. C3-4 synostosis was found incidentally in all patients on routine examination radiographs of cervical spine. RESULTS: Adjacent segment disease (ASD) was not found in the three patients younger than 39 years of age. Five of the 10 (50%) patients, including a 67-year-old man, did not develop spondylosis in any of the cervical mobile segments. Spondylosis was observed only in the caudal 1-2 mobile segments in the remaining five patients. The youngest was a 40-year-old male who had spondylosis in the two caudal mobile segments (C4-5 and C5-6). Spondylosis was limited to the two close caudal mobile segments and was not in the cranial segments. Flaring of the lower part of synostotic vertebra associated with advanced narrowed degenerate disc was evident in five patients. CONCLUSIONS: Mobile segment spondylosis in the individuals with congenital monosegment C3-4 synostosis over age of 40 years may be a natural manifestation of aging and is not solely an adjacent segment disease directly and fully related with congenital C3-4 synostosis.

Effectiveness of Bradycardia as a Single Parameter in the Pediatric Acute Response System / 대한구급학회지

BACKGROUND: Various tools for the acute response system (ARS) predict and prevent acute deterioration in pediatric patients. However, detailed criteria have not been clarified. Thus we evaluated the effectiveness of bradycardia as a single parameter in pediatric ARS. METHODS: This retrospective study included patients who had visited a tertiary care children's hospital from January 2012 to June 2013, in whom ARS was activated because of bradycardia. Patient's medical records were reviewed for clinical characteristics, cardiologic evaluations, and reversible causes that affect heart rate. RESULTS: Of 271 cases, 261 (96%) had ARS activation by bradycardia alone with favorable outcomes. Evaluations and interventions were performed in 165 (64.5%) and 13 cases (6.6%) respectively. All patients in whom ARS was activated owing to bradycardia and another criteria underwent evaluation, unlike those with bradycardia alone (100.0% vs. 63.2%, p = 0.016). Electrocardiograms were evaluated in 233 (86%) cases: arrhythmias were due to borderline QT prolongation and atrioventricular block (1st and 2nd-degree) in 25 cases (9.2%). Bradycardia-related causes were reversible in 202 patients (74.5%). Specific causes were different in departments at admission. Patients admitted to the hemato-oncology department required ARS activation during the night (69.3%, p = 0.03), those to the endocrinology department required ARS activation because of medication (72.4%, p < 0.001), and those to the gastroenterology department had low body mass indexes (32%, p = 0.01). CONCLUSIONS: Using bradycardia alone in pediatric ARS is not useful, because of its low specificity and poor predictive ability for deterioration. However, bradycardia can be applied to ARS concurrently with other parameters.

Kawasaki Disease with Acute Respiratory Distress Syndrome after Intravenous Immunoglobulin Infusion / 대한구급학회지

Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology. We report a case of KD with acute respiratory distress syndrome (ARDS) after intravenous immunoglobulin (IVIG) infusion. Lung manifestations associated with KD have previously been reported in the literature. Although IVIG infusion is an effective therapy for acute KD, there are some reported complications related to IVIG infusion: hypotension, aseptic meningitis, acute renal failure, hemolytic anemia, etc. The case of KD reported here was treated with IVIG and aspirin. A few days after recovery from KD, the patient developed fever and maculopapular rash. A diagnosis of relapse KD was made and retreated with IVIG infusion. However, the patient developed ARDS four days after the second IVIG infusion. The patient recovered from ARDS after nine days of ICU care, which included high frequency oscillation ventilation with inhaled nitric oxide, steroid treatment and other supportive care.

Radiographic Assessment of Effect of Congenital Monosegment Synostosis of Lower Cervical Spine between C2-C6 on Adjacent Mobile Segments

STUDY DESIGN: A prospective radiographic study of cervical spine with congenital monosegment fusion. PURPOSE: To evaluate the effect of cervical synostosis on adjacent segments and the vertebral morphology. OVERVIEW OF LITERATURE: There are numerous clinical studies of adjacent segment disease (ASD) after monosegment surgical fusion. However, there was no report on ASD in the cervical spine with congenital monosegment synostosis. METHODS: Radiograms of 52 patients, aged 5 to 90 years, with congenital monosegment synostosis (CMS) between C2 and C6, who complained of neck/shoulder discomfort or pain were studied. 51 were normally aligned and one was kyphotically aligned. RESULTS: Spondylosis was not found in the patients below 35 years of age. Only 12 out of 24 patients with normally aligned C2-3 synostosis had spondylosis in 19 more caudal segments, and only one at C3-4. A patient with kyphotic C2-3 had spondylolysis at C3-4. In 8 patients with C3-4 synostosis, spondylosis was found in only 9 caudal segments (4 at C4-5, 4 at C5-6, and 1 at C6-7). The caudate C4-5 disc was the most liable to degenerate in comparison with other caudate segments. Caudal corporal flaring and inwaisting of the synostotic vertebra were the features that were the most evident. In 2 of 9 C4-5 and 7 out of 10 C5-6 synostosis patients, spondylosis was found at the two adjacent cephalad and caudate segments, respectively. Only corporal inwaisting without flaring was found. In all cases, spondylosis was confined to the adjacent segments. More advanced spondylosis was found in the immediate caudal segment than the cephalad one. CONCLUSIONS: It is concluded that spondylosis at the mobile segments in a synostotic spine is thought to be a fusion-related pathology rather than solely age-related disc degeneration. Those data suggested that CMS definitely precipitated the disc degeneration in the adjacent segments.

Effectiveness of Bradycardia as a Single Parameter in the Pediatric Acute Response System / 대한중환자의학회지

BACKGROUND: Various tools for the acute response system (ARS) predict and prevent acute deterioration in pediatric patients. However, detailed criteria have not been clarified. Thus we evaluated the effectiveness of bradycardia as a single parameter in pediatric ARS. METHODS: This retrospective study included patients who had visited a tertiary care children's hospital from January 2012 to June 2013, in whom ARS was activated because of bradycardia. Patient's medical records were reviewed for clinical characteristics, cardiologic evaluations, and reversible causes that affect heart rate. RESULTS: Of 271 cases, 261 (96%) had ARS activation by bradycardia alone with favorable outcomes. Evaluations and interventions were performed in 165 (64.5%) and 13 cases (6.6%) respectively. All patients in whom ARS was activated owing to bradycardia and another criteria underwent evaluation, unlike those with bradycardia alone (100.0% vs. 63.2%, p = 0.016). Electrocardiograms were evaluated in 233 (86%) cases: arrhythmias were due to borderline QT prolongation and atrioventricular block (1st and 2nd-degree) in 25 cases (9.2%). Bradycardia-related causes were reversible in 202 patients (74.5%). Specific causes were different in departments at admission. Patients admitted to the hemato-oncology department required ARS activation during the night (69.3%, p = 0.03), those to the endocrinology department required ARS activation because of medication (72.4%, p < 0.001), and those to the gastroenterology department had low body mass indexes (32%, p = 0.01). CONCLUSIONS: Using bradycardia alone in pediatric ARS is not useful, because of its low specificity and poor predictive ability for deterioration. However, bradycardia can be applied to ARS concurrently with other parameters.

Kawasaki Disease with Acute Respiratory Distress Syndrome after Intravenous Immunoglobulin Infusion / 대한중환자의학회지

Kawasaki disease (KD) is an acute systemic vasculitis of unknown etiology. We report a case of KD with acute respiratory distress syndrome (ARDS) after intravenous immunoglobulin (IVIG) infusion. Lung manifestations associated with KD have previously been reported in the literature. Although IVIG infusion is an effective therapy for acute KD, there are some reported complications related to IVIG infusion: hypotension, aseptic meningitis, acute renal failure, hemolytic anemia, etc. The case of KD reported here was treated with IVIG and aspirin. A few days after recovery from KD, the patient developed fever and maculopapular rash. A diagnosis of relapse KD was made and retreated with IVIG infusion. However, the patient developed ARDS four days after the second IVIG infusion. The patient recovered from ARDS after nine days of ICU care, which included high frequency oscillation ventilation with inhaled nitric oxide, steroid treatment and other supportive care.

Effect of Correction of the Contractured Flexed Osteoarthritic Knee on the Sagittal Alignment by Total Replacement

STUDY DESIGN: A prospective analysis of an adaptive change of the spinopelvic alignment after total knee arthroplasty. PURPOSE: To evaluate the effect of correction of the contractured knee in flexion on the spinopelvic alignment by total knee arthroplasty. OVERVIEW OF LITERATURE: Flexion contracture of the knee joint may affect the body posture and precipitate the symptoms in the lumbar spine, which is known as the 'knee-spine syndrome'. METHODS: Fifteen patients who could be followed at least over 12 months were used in this study. Neutral whole spine lateral standing radiograms taken at certain intervals were analyzed. The subjects were divided into two groups (group A, the patients who obtained over 10degrees correction; group B, the others). The sacral slope, the pelvic tilt and the pelvic incidence were measured preoperatively and at 12 months and thereafter postoperatively in all the patients. Also, the thoracic kyphosis, lumbar lordosis, and lumbosacral angle were measured, including the spinal sagittal balance, S1 overhang and spino-sacral angle. RESULTS: The average correction of the contractured knee in flexion were 13.8degrees in group A and 2.7degrees in group B. The median of changes of the sacral slope were 4.2degrees in group A and -0.4degrees in group B. These results revealed that there was a significant increase of the sacral slope for group A (p=0.001). However, there were no significant differences between the other parameters. CONCLUSIONS: The sacral slope appears to be affected by the change of the flexion contracture after total knee arthroplasty.

Chronic Osteomyelitis of the Lumbar Transverse Process

Pyogenic spondylitis involving only the posterior element of a vertebra is rare. To the best of our knowledge, there have been no reports of osteomyelitis of the transverse process. We report here on a 45-year-old male with a one month history of swelling associated with lower back pain. The magnetic resonance imaging showed a paraspinal soft tissue mass, and computed tomography revealed a fine osteolytic lesion in the right transverse process of the 5th lumbar spine, and this was all consistent with chronic osteomyelitis. A mixed staphylococcal infection was identified. Open drainage, resection of the transverse process and intravenous injection of anti-staphylococcal antibiotics resolved the back pain and reduced the erythrocyte sedimentation rate to normal. Pyogenic osteomyelitis of the transverse process is extremely rare, which can cause a misdiagnosis or a delayed diagnosis. Careful consideration of this disease is needed when evaluating patients who complain of back pain.

Synoivial Chondromatosis of the Ankle Joint and Flexor Hallucis Longus Tendon Sheath / 대한족부족관절학회지

Synovial chondromatosis is a benign lesion forming multiple round cartilagenous nodules or osseous loose bodies in joint cavity. Predilection sites are known as knee, hip and elbow joints. However, the involvement of ankle joint was rarely reported in the literature. Moreover, extraarticular chondromatosis in synovial sheath or bursa of extremities is extremely rare. We present a case of synovial chondromatosis of the left ankle joint and flexor hallucis longus tendon sheath.

Hemiarthroplasty Using a Modular Hip Segment in a Patient with a Long Stem Total Knee Prosthesis

Femoral neck fractures frequently occur in elderly patients and the treatment strategies for this are well documented. Revision knee arthroplasty using a long intra-medullary stem has recently been increasingly used for treating complications such as infection, aseptic loosening or instability. The current case had a femoral neck fracture with a well-fixed, long stem knee prosthesis, which reached to near the lesser trochanter of the femur. The authors performed the surgery using impaction of the modular hip segment; its inner diameter was fit to the outer diameter of the femoral stem of the knee prosthesis. At two years postoperative follow-up, the result was excellent without loosening or osteolysis.

Low Grade Fibromyxoid Sarcoma in Thigh

A low grade fibromyxoid sarcoma is a rare soft tissue tumor that has a tendency to develop in the deep soft tissue of young adults and the potential for local recurrence or distant metastasis. There have been several case reports and sporadic reports in the literature. However, only 1 case has been reported in Korea but without a follow-up result. We describe a 54-year-old female patient with a low-grade fibromyxoid sarcoma of the thigh that had been growing slowly for 34 years. A marginal resection of this tumor was performed. Currently, the patient is doing well without evidence of local recurrence or distant metastasis at 5 years after surgery.

Lumbar Pyogenic Spondylodiscitis and Bilateral Psoas Abscesses Extending to the Gluteal Muscles and Intrapelvic Area Treated with CT-guided Percutaneous Drainage: A Case Report

Bilateral psoas abscesses extending to the gluteal muscle and intrapelvic area are uncommon. We present our experience with computed tomography (CT)-guided percutaneous catheter drainage for the treatment of multiple aggressive abscesses in a diabetic patient. The abscesses completely resolved after the procedures. Psoas abscess should be considered in the differential diagnosis of older diabetic patients with fever, flank or back pain, and flexion contracture of the hip joint. CT scanning is a useful method in diagnosing abscesses, and CT-guided percutaneous catheter drainage is an effective treatment method in selected patients.

Multiple Pyogenic Spondylodiscitis with Bilateral Psoas Abscesses Accompanying Osteomyelitis of Lateral Malleolus: A Case Report

A psoas abscess is a potentially life-threatening infection. Multiple pyogenic spondylodiscitis with bilateral psoas abscesses accompanying an osteomyelitis of the lateral malleolus is an extremely rare event. We present our experience with needle aspiration for the treatment of osteomyelitis of the lateral malleolus and CT-guided percutaneous catheter drainage for a psoas abscess in an elderly patient. Both infections were completely resolved without recurrence. A psoas abscess should be included in the differential diagnosis of a patient with low back pain during musculoskeletal infection. Percutaneous needle aspiration or CT-guided percutaneous catheter drainage is an effective method for treating certain musculoskeletal infections.

LumbarWedgeResectionOsteotomyforCongenitalScoliosis duetoaSacralMalformation: A CaseReport / 대한정형외과학회잡지

Congenital scoliosis due to a sacral malformation is quite rare. To the best of our knowledge, most wedge resection osteotomies have been performed to correct a kyphotic deformity in ankylosing spondylitis. However, there is no report of a trapezoidal lumbar wedge resection osteotomy of the vertebral body in the surgical treatment of congenital scoliosis due to a sacral malformation. This paper reports a 41-year-old female with a 25-year history of lower back and buttock pain combined with radiating pain to the lower extremities. The coronal imbalance was 3.8 cm and the scoliosis angle using the Cobb method was 22 degrees. A trapezoidal wedge resection osteotomy of the L5 body was performed, and the scoliosis was corrected. We detail this modification of a vertebral osteotomy technique and show that a fixed coronal deformity could be corrected effectively using this technique.

Cemented Bipolar Hemiarthroplasty for the Femoal Neckor Intertrochanteric Fracture in Elderly Patients: Multicenter Study / 대한정형외과학회잡지

Purpose: This multicenter study was carried out to estimate the clinical and radiological results of cemented bipolar endoprosthesis replacement arthroplasty of a hip fracture in patients aged over 50 years in Jeju Island. Materials and Methods: Eighty-eight hips underwent bipolar endoprosthesis in 2002. Thirty-two patients died during the follow-up period and 7 patients refused a radiological evaluation or an interview. Two patients who underwent cementless bipolar endoprosthesis were excluded. Forty- nine hips (48 patients) completed the average 27 months follow-up. A neck fracture was involved in 40 hips (39 patient) and an intertrochanteric fracture was involved in 9 hips (9 patients). The average age was 77.7 years. The results were evaluated using clinical and radiological methods. Results: At the last follow-up, the average Harris hip score was 70.1 and 22 hips (45%), which indicated excellent and good results, respectively. Among the 42 patients with normal activity at the preoperative period, 28 patients were still active at the final follow-up. The number of medical illnesses and the severity of mental deterioration were also associated with the poor Harris hip scores. The pressurization cementing technique was found to be excellent at the radiological evaluation. One femoral stem appeared to have loosened and subsided but there was no revision surgery. Conclusion: The clinical result of the cemented bipolar endoprosthesis in multicenter study showed that the only 45% of patients showed results >good. The major factors influencing the results were the postoperative activity, the number of medical illnesses, and the severity of the mental changes.

Spinal Epidural Abscess with Pyogenic Arthritis of Facet Joint Treated with Antibiotic-Bone Cement Beads: A Case Report

Most epidural abscesses are a secondary lesion of pyogenic spondylodiscitis. An epidural abscess associated with pyogenic arthritis of the facet joint is quite rare. To the best of our knowledge, there is no report of the use of antibiotic-cement beads in the surgical treatment of an epidural abscess. This paper reports a 63-year-old male who sustained a 1-week history of radiating pain to both lower extremities combined with lower back pain. MRI revealed space-occupying lesions, which were located in both sides of the anterior epidural space of L4, and CT scans showed irregular widening and bony erosion of the facet joints of L4-5. A staphylococcal infection was identified after a posterior decompression and an open drainage. Antibiotic- bone cement beads were used as a local controller of the infection and as a spacer or an indicator for the second operation. An intravenous injection of anti-staphylococcal antibiotics resolved the back pain and radicular pain and normalized the laboratory findings. We point out not only the association of an epidural abscess with facet joint infection, but also the possible indication of antibiotic-bone cement beads in the treatment of epidural abscesses.

The Effect of Indwelling Silk Suture Following Aspiration in the Treatment of Chronic Lateral Malleolar Bursitis / 대한족부족관절학회지

PURPOSE: To evaluate the drainage effect of silk suture following aspiration of the bursa as an early treatment of chronic lateral malleolar bursitis MATERIALS AND METHODS: Thirteen cases, which have over two weeks of history and over one year of follow-up, were investigated. The average duration of follow-up was 16.4 months. The average symptom duration before introduction into this study was 7.8 weeks. With an aseptic technique, the aspiration of the bursa was done with 18G needle and syringe and then the insertion of silk suture through the aspiration needle was performed. The amount of drainage was identified two or three times in a week and stitch out was done at the cessation of drainage. Over one year follow-up, recurrence, infection, pain, and limitation of range of motion were investigated by telephone interview. RESULTS: Redness around the insertion site of silk suture was found in all cases, but there was no development of active infection or recurrence. The average duration of treatment is 10.4 days. CONCLUSION: The drainage with silk suture following aspiration of the bursa is less invasive and very effective method in the early treatment of chronic lateral malleolar bursitis.