Membranoproliferative glomerulonephritis type II(MPGN II), also called dense deposit disease, was first described by Berger and Galle in 1963. The diagnosis of MPGN II is based on electron-microscopic finding of an intensely electron-dense substance which replaces the lamina densa of the glomerular basement membrane. Although the etiology and pathogenesis of MPGN II are unknown, it frequently progresses to end-stage renal failure. Typically in MPGN II, hypocomplementemia due to activation of the alternative complement pathway is present. In addition, the association of MPGN II with partial lipodystrophy and complement abnormalities is well documented. The relationship between these associated features and the patient's renal functional outcome is not clear. With respect to the therapy for MPGN II, an alternate-day prednisolone regimen was shown to be effective. Various treatment modalities, including immunosuppression with corticosteroids, cytotoxic drugs and cyclosporin A, anticoagulants and antiplatelet therapies are used, either alone or in combination, with varying degrees of success. The purpose of this paper is to present a case of MPGN II from a 7 years old girl with paroxysmal supraventricular tachycardia(PSVT).
Adrenal Cortex Hormones ; Anticoagulants ; Child ; Complement Pathway, Alternative ; Complement System Proteins ; Cyclosporine ; Diagnosis ; Female ; Glomerular Basement Membrane ; Glomerulonephritis, Membranoproliferative* ; Humans ; Immunosuppression ; Kidney Failure, Chronic ; Lipodystrophy ; Prednisolone

Membranoproliferative glomerulonephritis type II(MPGN II), also called dense deposit disease, was first described by Berger and Galle in 1963. The diagnosis of MPGN II is based on electron-microscopic finding of an intensely electron-dense substance which replaces the lamina densa of the glomerular basement membrane. Although the etiology and pathogenesis of MPGN II are unknown, it frequently progresses to end-stage renal failure. Typically in MPGN II, hypocomplementemia due to activation of the alternative complement pathway is present. In addition, the association of MPGN II with partial lipodystrophy and complement abnormalities is well documented. The relationship between these associated features and the patient's renal functional outcome is not clear. With respect to the therapy for MPGN II, an alternate-day prednisolone regimen was shown to be effective. Various treatment modalities, including immunosuppression with corticosteroids, cytotoxic drugs and cyclosporin A, anticoagulants and antiplatelet therapies are used, either alone or in combination, with varying degrees of success. The purpose of this paper is to present a case of MPGN II from a 7 years old girl with paroxysmal supraventricular tachycardia(PSVT).
Adrenal Cortex Hormones ; Anticoagulants ; Child ; Complement Pathway, Alternative ; Complement System Proteins ; Cyclosporine ; Diagnosis ; Female ; Glomerular Basement Membrane ; Glomerulonephritis, Membranoproliferative* ; Humans ; Immunosuppression ; Kidney Failure, Chronic ; Lipodystrophy ; Prednisolone

Turner syndrome is well known to be associated with significant cardiovascular abnormalities. This paper studied the incidence of cardiovascular abnormalities in asymptomatic adolescent patients with Turner syndrome using multidetector computed tomography (MDCT) instead of echocardiography. Twenty subjects diagnosed with Turner syndrome who had no cardiac symptoms were included. Blood pressure and electrocardiography (ECG) was checked. Cardiovascular abnormalities were checked by MDCT. According to the ECG results, 11 had a prolonged QTc interval, 5 had a posterior fascicular block, 3 had a ventricular conduction disorder. MDCT revealed vascular abnormalities in 13 patients (65%). Three patients had an aberrant right subclavian artery, 2 had dilatation of left subclavian artery, and others had an aortic root dilatation, aortic diverticulum, and abnormal left vertebral artery. As for venous abnormalities, 3 patients had partial anomalous pulmonary venous return and 2 had a persistent left superior vena cava. This study found cardiovascular abnormalities in 65% of asymptomatic Turner syndrome patients using MDCT. Even though, there are no cardiac symptoms in Turner syndrome patients, a complete evaluation of the heart with echocardiography or MDCT at transition period to adults must be performed.
Adolescent ; Blood Pressure ; Cardiovascular Abnormalities/complications/epidemiology ; Electrocardiography ; Humans ; Karyotyping ; Multidetector Computed Tomography ; Prevalence ; Turner Syndrome/complications/*diagnosis/radiography ; Vascular Malformations/complications/epidemiology ; Vertebral Artery/abnormalities ; Young Adult

Cranial nerve palsies are relatively common after trauma, but trochlear nerve palsy is relatively uncommon. Although traumatic trochlear nerve palsy is easy to diagnose clinically because of extraocular movement disturbances, radiologic evaluations of this condition are difficult to perform because of the nerve's small size. Here, we report the case of a patient with delayed traumatic trochlear nerve palsy associated with a traumatic subarachnoid hemorrhage (SAH) and the related radiological findings, as obtained with high-resolution three-dimensional (3D) magnetic resonance imaging (MRI). A 63-year-old woman was brought to the emergency room after a minor head trauma. Neurologic examinations did not reveal any focal neurologic deficits. Brain computed tomography showed a traumatic SAH at the left ambient cistern. The patient complained of vertical diplopia at 3 days post-trauma. Ophthalmologic evaluations revealed trochlear nerve palsy on the left side. High-resolution 3D MRI, performed 20 days post-trauma, revealed continuity of the trochlear nerve and its abutted course by the posterior cerebral artery branch at the brain stem. Chemical irritation due to the SAH and the abutting nerve course were considered causative factors. The trochlear nerve palsy completely resolved during follow-up. This case shows the usefulness of high-resolution 3D MRI for evaluating trochlear nerve palsy.
Brain ; Brain Stem ; Cranial Nerve Diseases ; Craniocerebral Trauma ; Diplopia ; Emergency Service, Hospital ; Female ; Follow-Up Studies ; Humans ; Imaging, Three-Dimensional ; Magnetic Resonance Imaging* ; Middle Aged ; Neurologic Examination ; Neurologic Manifestations ; Posterior Cerebral Artery ; Subarachnoid Hemorrhage, Traumatic* ; Trochlear Nerve Diseases* ; Trochlear Nerve*

Carpal tunnel syndrome(CTS), the most common compressive neuropathy, is usually diagnosed by clinical features and nerve conduction test(NCS). However, NCS might show no abnormal finding. Ultrasonography(USG), known as helpful adjunctive in diagnosis of CTS, also might show false negative finding. A 33-year-old woman presented with complaints of pain and numbness in median nerve area on her right hand for 4 years. Despite typical clinical features of CTS, neither NCS nor USG showed abnormal finding. Because of persistent symptom, without significant improvement on conservative management, endoscopic carpal tunnel release(ECTR) was performed with carpal tunnel pressure(CTP) measurement. The measured CTP was 27.9mmHg before ECRT, which was reduced to 5.9mmHg after operation. The pain and numbness subsided after operation. Our case showed the usefulness of CTP measurement in diagnosis of CTS. The measurement of CTP might be an important diagnosis modality for some patients having CTS, especially in cases without definitive findings in NCS and USG.
Adult ; Carpal Tunnel Syndrome ; Cytidine Triphosphate ; Electromyography ; Female ; Hand ; Humans ; Hypesthesia ; Median Nerve ; Neural Conduction

OBJECTIVE: This study was done to evaluate the correlation between carpal tunnel pressure (CTP), electrodiagnostic and ultrasonographic findings in patients with carpal tunnel syndrome (CTS). METHODS: CTP was measured during endoscopic carpal tunnel release (ECTR) for CTS using Spiegelberg ICP monitoring device with parenchymal type catheter. Neurophysiologic severity and nerve cross sectional area were evaluated using nerve conductive study and ultrasonography (USG) before ECTR in all patients. RESULTS: Tests were performed in a total of 48 wrists in 39 patients (9 cases bilateral). Maximum CTP was 56.7 +/- 19.3 mmHg (Mean +/- SD) and 7.4 +/- 3.3 mmHg before and after ECTR, respectively. No correlation was found between maximum CTP and either neurophysiologic severity or nerve cross sectional area, whereas we found a significant correlation between the latter two parameters. CONCLUSION: CTP was not correlated with neurophysiologic severity and nerve cross sectional area. Dynamic, rather than static, pressure in carpal tunnel might account for the basic pathophysiology of CTS better.
Carpal Tunnel Syndrome ; Catheters ; Cytidine Triphosphate ; Electrodiagnosis ; Humans ; Wrist

OBJECTIVE: Adequate management of increased intracranial pressure (ICP) is critical in patients with traumatic brain injury (TBI), and decompressive craniectomy is widely used to treat refractory increased ICP. The authors reviewed and analyzed complications following decompressive craniectomy for the management of TBI. METHODS: A total of 89 consecutive patients who underwent decompressive craniectomy for TBI between February 2004 and February 2009 were reviewed retrospectively. Incidence rates of complications secondary to decompressive craniectomy were determined, and analyses were performed to identify clinical factors associated with the development of complications and the poor outcome. RESULTS: Complications secondary to decompressive craniectomy occurred in 48 of the 89 (53.9%) patients. Furthermore, these complications occurred in a sequential fashion at specific times after surgical intervention; cerebral contusion expansion (2.2 +/- 1.2 days), newly appearing subdural or epidural hematoma contralateral to the craniectomy defect (1.5 +/- 0.9 days), epilepsy (2.7 +/- 1.5 days), cerebrospinal fluid leakage through the scalp incision (7.0 +/- 4.2 days), and external cerebral herniation (5.5 +/- 3.3 days). Subdural effusion (10.8 +/- 5.2 days) and postoperative infection (9.8 +/- 3.1 days) developed between one and four weeks postoperatively. Trephined and post-traumatic hydrocephalus syndromes developed after one month postoperatively (at 79.5 +/- 23.6 and 49.2 +/- 14.1 days, respectively). CONCLUSION: A poor GCS score (< or = 8) and an age of > or = 65 were found to be related to the occurrence of one of the above-mentioned complications. These results should help neurosurgeons anticipate these complications, to adopt management strategies that reduce the risks of complications, and to improve clinical outcomes.
Brain Injuries ; Contusions ; Decompressive Craniectomy ; Epilepsy ; Hematoma ; Humans ; Hydrocephalus ; Incidence ; Intracranial Pressure ; Retrospective Studies ; Scalp ; Subdural Effusion

BACKGROUND: Cancer rates are increasing not only in the general population but also in patients with end-stage renal disease. We investigated the changing pattern of pretransplant malignancy in kidney transplant recipients over 5 decades.METHODS: We reviewed 3,748 kidney transplant recipients between 1969 and 2016. We divided patients into three groups (1969–1998, 1999–2006, 2007–2016) based on the era of the cancer screening system used throughout the nation. We analyzed the incidence and pattern of pretransplant malignancy among the three groups. We also evaluated recurrent and de novo malignancy in these patients compared to patients without pretransplant malignancy.RESULTS: A total of 72 patients exhibited pretransplant malignancy (1.9%). There were no cases of pretransplant cancer until 1998, but the rate of pretransplant malignancy gradually increased to 1.1% during 1999–2006 and further increased to 4.3% thereafter. The most frequent types of pretransplant malignancy changed from the bladder, liver, and stomach cancers to thyroid cancer and renal cell carcinoma. There were no de novo cases, but there were three cases of recurrent cancer in patients with pretransplant malignancy; the recurrence rate among kidney transplant recipients with pretransplant malignancy was not significantly different from the incidence rate of de novo malignancy among kidney transplant recipients without pretransplant malignancy (4.2% vs. 6.9%, P = 0.48).CONCLUSION: The incidence of pretransplant malignancy in kidney transplantation candidates is gradually increasing, and recent increases were accompanied by changes in cancer types. Pretransplant malignancy may not be a hindrance to kidney transplantation because of the low incidence of posttransplant recurrence and de novo malignancy.
Carcinoma, Renal Cell ; Early Detection of Cancer ; Humans ; Incidence ; Kidney Failure, Chronic ; Kidney Transplantation ; Kidney ; Liver ; Recurrence ; Stomach Neoplasms ; Thyroid Neoplasms ; Transplant Recipients ; Urinary Bladder