Skeletal stability after 2-jaw surgery via surgery-first approach in facial asymmetry patients using CBCT / 대한악안면성형재건외과학회지

Background@#The purpose of this study is to compare the skeletal stability of two-jaw surgery via surgery-first approach with conventional two-jaw surgery in facial asymmetry patients by measuring the skeletal changes after surgery from a three-dimensional analysis. From January 2010 to January 2014, 40 patients with facial asymmetry who underwent two-jaw surgery in Pusan National University Hospital were included in this study. They were classified into experimental group (n = 20) who underwent two-jaw surgery via surgery-first approach and control group (n = 20) who underwent conventional two-jaw surgery. After selection of 24 landmarks and the construction of horizontal and sagittal, coronal reference planes, changes in 10 linear measurements and 2 angular measurements were compared between the surgery-first approach and conventional groups in the preoperative, immediate postoperative, and postoperative periods. The paired t test and Student t test were used for statistical analysis. The mean and standard deviation of the measurement were calculated for the experimental and control groups. @*Results@#The statistical analysis showed that changes in skeletal measurements were similar between the surgery-first approach and conventional groups, according to each period. However, U1-SRP measurement showed statistically significant changes in surgery-first approach groups at postsurgical change (T1 to T2). Also, the mean treatment duration in the treatment group was 15.9 ± 5.48 months whereas that in the control group was 32.9 ± 14.05months. @*Conclusion@#In facial asymmetry patients, similar results were observed in the postoperative skeletal stability when 2- jaw surgery via surgery-first approach was compared with conventional 2-jaw surgery. However, significant lateral deviation of upper incisor midline was observed. In addition, a shorter average treatment duration was observed. To stabilize the unstable occlusion after surgery, increased wearing of the stent and proactive rubber guidance will be needed.

Re-transmissibility of mouse-adapted ME7 scrapie strain to ovine PrP transgenic mice

Scrapie is a mammalian transmissible spongiform encephalopathy or prion disease that predominantly affects sheep and goats. Scrapie has been shown to overcome the species barrier via experimental infection of other rodents. To confirm the re-transmissibility of the mouse-adapted ME7 scrapie strain to ovine prion protein (PrP) transgenic mice, mice of an ovinized transgenic mouse line carrying the Suffolk sheep PrP gene that contained the A₁₃₆ R₁₅₄ Q₁₇₁/ARQ allele were intracerebrally inoculated with brain homogenates obtained from terminally ill ME7-infected C57BL/6J mice. Herein, we report that the mouse-adapted ME7 scrapie strain was successfully re-transmitted to the transgenic mice expressing ovine PrP. In addition, we observed changes in the incubation period, glycoform profile, and pattern of scrapie PrP (PrP(Sc)) deposition in the affected brains. PrP(Sc) deposition in the hippocampal region of the brain of 2nd-passaged ovine PrP transgenic mice was accompanied by plaque formation. These results reveal that the mouse-adapted ME7 scrapie strain has the capacity to act as a template for the conversion of ovine normal monomeric precursors into a pathogenic form in ovine PrP transgenic mice. The change in glycoform pattern and the deposition of plaques in the hippocampal region of the brain of the 2nd-passaged PrP transgenic mice are most likely cellular PrP species dependent rather than being ME7 scrapie strain encoded.

Fatal vocal cord granuloma after orthognathic surgery

Endotracheal intubation is commonly associated with laryngeal injury that often resolves spontaneously without any complication. However, stenosis or granulomatous lesions are generally found on the tracheal wall or vocal process at the tube cuff level, caused by excessive cuff pressure. We present a case of fatal vocal cord granuloma leading to dyspnea following orthognathic surgery and sustained intubation for 14 hours.

Novel Mutation in PTHLH Related to Brachydactyly Type E2 Initially Confused with Unclassical Pseudopseudohypoparathyroidism

BACKGROUND: Autosomal-dominant brachydactyly type E is a congenital abnormality characterized by small hands and feet, which is a consequence of shortened metacarpals and metatarsals. We recently encountered a young gentleman exhibiting shortening of 4th and 5th fingers and toes. Initially, we suspected him having pseudopseudohypoparathyroidism (PPHP) because of normal biochemical parameters, including electrolyte, Ca, P, and parathyroid hormone (PTH) levels; however, his mother and maternal grandmother had the same conditions in their hands and feet. Furthermore, his mother showed normal biochemical parameters. To the best of our knowledge, PPHP is inherited via a mutated paternal allele, owing to the paternal imprinting of GNAS (guanine nucleotide binding protein, alpha stimulating) in the renal proximal tubule. Therefore, we decided to further analyze the genetic background in this family. METHODS: Whole exome sequencing was performed using genomic DNA from the affected mother, son, and the unaffected father as a negative control. RESULTS: We selected the intersection between 45,490 variants from the mother and 45,646 variants from the son and excluded 27,512 overlapping variants identified from the father. By excluding homogenous and compound heterozygous variants and removing all previously reported variants, 147 variants were identified to be shared by the mother and son. Variants that had least proximities among species were excluded and finally 23 variants remained. CONCLUSION: Among them, we identified a defect in parathyroid hormone like hormone (PTHLH), encoding the PTH-related protein, to be disease-causative. Herein, we report a family affected with brachydactyly type E2 caused by a novel PTHLH mutation, which was confused with PPHP with unclassical genetic penetrance.

Versatility of the pedicled buccal fat pad flap for the management of oroantral fistula: a retrospective study of 25 cases / 대한악안면성형재건외과학회지

PURPOSE@#Maxillary bone grafts and implantations have increased over recent years despite a lack of maxillary bone quality and quantity. The number of patients referred for oroantral fistula (OAF) due to implant or bone graft failure has increased, and in patients with an oroantral fistula, the pedicled buccal fat pad is viewed as a robust, reliable option. This study was conducted to document the usefulness of buccal fat pad grafts for oroantral fistula closure.@*MATERIALS AND METHODS@#We retrospectively studied 25 patients with OAF treated with a buccal fat pad graft from 2015 to 2018. Sex, age, OAF location, cause, duration, presence of systemic disease, smoking, previous dental surgery, and side effects were investigated.@*RESULTS@#A total of 25 patients were studied. Mean patient age was 54.8 years, and the male to female ratio was 19:6. Causes of oroantral fistula were cyst enucleation, tumor resection, implant removal, bone graft failure, and extraction. Excellent results were obtained in 23 (92%) of the 25 patients. In the other two patients that both smoked, a small fistula was observed during follow-up. No recurrence of oroantral fistula was observed after 2 months to 1 year of follow-up.@*CONCLUSIONS@#The incidence of oroantral fistula is increasing due to implant and bone graft failures. Oroantral fistula closure using a pedicled buccal fat pad was found to have a high success rate.

Diarylbutane-type Lignans from Myristica fragrans (Nutmeg) show the Cytotoxicity against Breast Cancer Cells through Activation of AMP-activated Protein Kinase

In our program to search for new AMP-activated protein kinase (AMPK) activators from plants that exert potential anticancer property, we found that an EtOAc extract of Myristica fragrans (nutmeg) activated AMPK enzyme in human breast cancer MCF-7 cells. Two major diarylbutane-type lignans, macelignan and meso-dihydroguaiaretic acid (MDGA), were isolated as active principles from this extract. Treatment of breast cancer cells with two compounds induced cellular apoptosis, evidenced by cleavage of poly-(ADP-ribose) polymerase (PARP) and Ser 15 phosphorylation of p53. Moreover, macelignan and MDGA significantly inhibited the colony formation of MCF-7 breast cancer cells on soft agar. Intraperitoneal injection of macelignan and MDGA (20 mg/kg) suppressed the tumor growth of 4T1 mammary cancer cells. These results indicate that the chemopreventive effects of two major diarylbutane-type lignans from Myristica fragrans (nutmeg) may be associated with induction of apoptosis presumably through AMPK activation.

The rate and stability of mandibular block bone graft in recent 5 years / 대한악안면성형재건외과학회지

BACKGROUND: The purposes of the present study were to compare implant stabilities of mandibular block bone graft and bovine bone graft and to evaluate influencing factors for implant stability in mandibular block bone (MBB) graft. METHODS: This retrospective study investigated 1224 cases and 389 patients treated by one surgeon in the Department of Oral and Maxillofacial Surgery of Pusan National University Dental Hospital (Yangsan, Korea) between January 2010 and December 2014. Proportions that MBB graft cases constitute in all implant restoration cases and in all bone graft cases were measured. Implant stability quotient (ISQ) values were achieved by the same surgeon before loading. The average ISQ values of the experimental groups were compared. In addition, ISQ values of influencing factors, such as age, sex, implant size, and implant placement site, were compared within the MBB group using OsstellTM Mentor (Osstell®, Göteborg, Sweden). Paired t test and ANOVA were conducted for statistical analysis with a significance level of 0.05. RESULTS: Fifty-five percent of all implant restoration cases performed bone graft while MBB cases constituted 34% of all implant restoration cases and 61% of all bone graft cases. Comparing ISQ values according to bone graft materials, the MBB group manifested sufficient implant stability by presenting comparable ISQ value to that of the experimental group without bone graft. Among the reviewed factors, females, mandibular molar regions, and implants in larger diameter displayed greater implant stabilities. CONCLUSIONS: Satisfactory implant stability was accomplished upon administration of MBB graft. Within the limitation of this study, gender, implant site, and implant diameter were speculated to influence on implant stability in MBB graft.

Real-Time Quaking-Induced Conversion Analysis for the Diagnosis of Sporadic Creutzfeldt-Jakob Disease in Korea

BACKGROUND AND PURPOSE: The level of 14-3-3 protein in the cerebrospinal fluid (CSF) is increased in Creutzfeldt-Jakob disease (CJD) patients, which has led to it being used as a clinical biomarker for the ante-mortem diagnosis of human prion diseases. However, the specificity of the 14-3-3 protein is less reliable for CJD diagnosis. Newly developed assays including real-time quaking-induced conversion (RT-QuIC) have made it possible to detect the PrPSc-like abnormal prion isoform with a high sensitivity in animal and human specimens that might contain a minute amount of PrP(Sc) due to in vitro prion replication. METHODS: This study applied a highly sensitive RT-QuIC assay using recombinant human PrP to detect PrP(Sc) in the CSF of 81 patients with sporadic CJD (sCJD) in Korea. RESULTS: RT-QuIC analysis of the CSF samples based on the expression levels of 14-3-3 and total tau proteins revealed positivity in 62 of 81 sCJD patients (sensitivity of 76.5%) but no positive results in the 100 non-CJD patients. CONCLUSIONS: The sensitivity of the RT-QuIC in this study was similar to that in some previous reports, and the specificity of RT-QuIC was higher than that of 14-3-3 in CSF, suggesting that RT-QuIC analysis can complement the weakness of the specificity of 14-3-3 for the diagnosis of sCJD. These results indicate that RT-QuIC might be very useful for the rapid and specific diagnosis of sCJD and provide a practical novel method for the ante-mortem diagnosis of human prion diseases.

Pharyngeal flap damage caused by nasotracheal intubation in a patient who underwent palatoplasty and pharyngoplasty

Patients with cleft lip and palate (CLP) must undergo corrective surgeries during infancy and early childhood. Many patients with CLP undergo orthognathic surgery during their childhood for correction of skeletal asymmetries or pharyngoplasty with a pharyngeal flap to improve the quality of speech and velopharyngeal function. During orthognathic surgeries, nasotracheal intubation is performed under general anesthesia. In our case report, the patient had undergone palatoplasty and pharygoplasty previously. During the orthognathic surgery, a flexible fiberoptic bronchoscope-guided nasotracheal tube was inserted through the pharyngeal flap ostium; however, active bleeding occurred in the nasopharynx. Bleeding occurred because the flap was torn. After achieving hemostasis, the surgery was completed successfully. Thus, if a patient may show the potential for velopharyngeal port obstruction, nasotracheal intubation should be performed with utmost care.

Difficult airway management in a patient with a thin mandible

A 47-year-old woman was referred for surgical treatment of osteomyelitis of the mandible. She had already undergone three previous surgeries. Pre-anesthetic airway evaluation predicted a difficult airway, due to the thin, retro-positioned mandible, tongue, and atrophic changes in the lips and soft tissue. We inserted packing gauzes in the buccal mucosa for easier mask fitting and ventilation. During direct laryngoscopic intubation with a nasotracheal tube (NTT), fracture of a thin mandible can easily occur. Therefore, we used a fiberoptic bronchoscope to insert the NTT. After surgery, we performed a tongue-tie to protect against airway obstruction caused by the backward movement of the tongue during recovery. The patient recovered without any complications. We determined the status of the patient precisely and consequently performed thorough preparations for the surgery, allowing the patient to be anesthetized safely and recover after surgery. Careful assessment of the patient and airway prior to surgery is necessary.

HBV reactivation in a HBsAg-negative patient with multiple myeloma treated with prednisolone maintenance therapy after autologous HSCT

Hepatitis B virus (HBV) reactivation has previously occurred in hepatitis B surface antigen-negative patients with malignant lymphoma who received rituximab-based combination chemotherapy. However, few reports have described cases of HBV reactivation in patients with multiple myeloma thus far. We report a case of HBV reactivation in a patient with multiple myeloma treated with chemotherapy, autologous hematopoietic stem cell transplantation, and maintenance steroid therapy. For the HBV reactivation, the patient was treated with the antiviral agent entecavir. The clinical symptoms and laboratory findings improved after 3 months. Further studies should target the identification of patients at high risk of HBV reactivation in multiple myeloma treated with autologous hematopoietic stem cell transplantation and steroid therapy for maintenance and establish viral prophylaxis strategies, especially in Korea, in which HBV infection is endemic.

Laparoscopic uterine artery occlusion before cervical curettage in cervical ectopic pregnancy: Safe and effective for preventing massive bleeding

Cervical ectopic pregnancy is associated with high risk for massive bleeding conditions. Cervical ectopic pregnancy can usually be treated by methotrexate injection or surgery. We present 4 cases of cervical ectopic pregnancy that were treated successfully with different uterine-conserving methods. By comparing our experience of 4 cases managed in different ways, we found that laparoscopic uterine artery occlusion before cervical curettage is more effective method for preventing massive bleeding.

Medullary Thyroid Carcinoma with Ectopic Adrenocorticotropic Hormone Syndrome

Ectopic adrenocorticotropic hormone (ACTH) syndrome is caused most frequently by a bronchial carcinoid tumor or by small cell lung cancer. Medullary thyroid carcinoma (MTC) is a rare etiology of ectopic ACTH syndrome. We describe a case of Cushing syndrome due to ectopic ACTH production from MTC in a 48-year-old male. He was diagnosed with MTC 14 years ago and underwent total thyroidectomy, cervical lymph node dissection and a series of metastasectomies. MTC was confirmed by the pathological examination of the thyroid and metastatic mediastinal lymph node tissues. Two years after his last surgery, he developed Cushingoid features, such as moon face and central obesity, accompanied by uncontrolled hypertension and new-onset diabetes. The laboratory results were compatible with ectopic ACTH syndrome. A bilateral adrenalectomy improved the clinical and laboratory findings that were associated with Cushing syndrome. This is the first confirmed case of ectopic ACTH syndrome caused by MTC in Korea.

Surgical versus Conservative Treatment for Lumbar Disc Herniation with Motor Weakness

OBJECTIVE: The aim of this study is to assess outcomes during first one year for patients with severe motor weakness caused by lumbar disc herniation that underwent surgical or nonsurgical treatment. METHODS: The 46 patients with motor weakness because of lumbar disc herniation who were treated at neurosurgical department and rehabilitation in our hospital from 2006 to 2010, retrospectively. Each group had 26 surgical treatments and 20 conservative treatments. We followed up 1, 3, 6 months and 12 month and monitored a Visual Analogue rating Scale (VAS) of back and leg pain, Oswestry Disability Index (ODI) and degree of motor weakness. We analyzed the differences between surgical and nonsurgical groups using Mann-Whitney U test and repeat measure ANOVA in each follow-up periods. RESULTS: In the recovery of motor weakness, surgical treatment uncovered a rapid functional recovery in the early periods (p=0.003) and no difference between groups at the end of follow-up period was found (p>0.05). In VAS of back and leg, the interaction between time and group was not found (p>0.05) and there was no difference between groups (p>0.05). In ODI, the interaction between time and group was not found (p>0.05) and there was no difference between groups (p>0.05). CONCLUSION: Surgical treatment for motor weakness caused by herniated intervertebral disc resulted in a rapid recovery in the short-term period, especially 1 month. We think early and proper surgical treatment in a case of motor weakness from disc herniation could be a good way for providing a chance for rapid alleviation.

Incidental Superior Hypophygeal Artery Aneurysm Embedded within Pituitary Adenoma

Intra-cranial aneurysm can be incidental findings in patients with pituitary adenomas, and are usually located outside the pituitary region. However, the coexistence of intrasellar (not intracranial) aneurysms with pituitary adenomas is extremely rare. We report a patient with an incidental superior hypophygeal aneurysm embedded within a non-functional pituitary adenoma which was treated by transsphenoidal surgery after endovascular coil embolization.

Hypoglycemic Seizures Associated with Pentamidine for Pneumocystis jiroveci Pneumonia in a HIV Patient / 대한내과학회지

Trimethoprim-sulfamethoxazole (TMX-SMZ) is the initial treatment for Pneumocystis jiroveci pneumonia in human immunodeficiency virus (HIV) patients. About 20% of patients do not complete the TMX-SMZ treatment due to treatment failure or adverse reactions. Pentamidine isethionate has been used for P. jiroveci pneumonia as a second-line regimen. Although hypoglycemia is a common adverse effect of pentamidine, pentamidine-induced hypoglycemia has not been reported in Korea. We present an HIV patient with grand mal seizures caused by pentamidine-induced hypoglycemia who was managed successfully with IV dextrose infusion. Mental changes can occur during pentamidine treatment, but hypoglycemia is often ignored and misdiagnosed as epilepsy or stroke. It can result in seizures, coma, and even death. We should be aware of pentamidine-induced hypoglycemia, which can lead to severe neurologic deficits and diabetes mellitus.

Acute oxalate nephropathy caused by ethylene glycol poisoning

Ethylene glycol (EG) is a sweet-tasting, odorless organic solvent found in many agents, such as anti-freeze. EG is composed of four organic acids: glycoaldehyde, glycolic acid, glyoxylic acid and oxalic acid in vivo. These metabolites are cellular toxins that can cause cardio-pulmonary failure, life-threatening metabolic acidosis, central nervous system depression, and kidney injury. Oxalic acid is the end product of EG, which can precipitate to crystals of calcium oxalate monohydrate in the tubular lumen and has been linked to acute kidney injury. We report a case of EG-induced oxalate nephropathy, with the diagnosis confirmed by kidney biopsy, which showed acute tubular injury of the kidneys with extensive intracellular and intraluminal calcium oxalate monohydrate crystal depositions.

A Case of P-ANCA-Positive Anti-Glomerular Basement Membrane Antibody Disease / 대한내과학회지

Up to 40% of patients with anti-glomerular basement membrane (GBM) disease, which is a rare autoimmune disorder usually manifesting as rapidly progressive glomerulonephritis (RPGN), are positive for circulating anti-neutrophil cytoplasmic antibody (ANCA). Many previous reports showed poor outcomes in these "double-positive" patients. We report a patient with perinuclear (p)-ANCA positive anti-GBM disease who presented with RPGN and required hemodialysis. Plasmapheresis and steroid and cyclophosphamide therapy were initiated following renal biopsy and resulted in normalization of anti-GBM antibody and p-ANCA titers, recovery of renal function, and discontinuation of hemodialysis. This case suggests that aggressive immunosuppression with plasmapheresis in patients who are p-ANCA positive with anti-GBM disease should be considered, even in those with severe renal dysfunction.

A Case of Giant Cell Arteritis Accompanied by Acute Myeloid Leukemia

Giant cell arteritis (GCA), the most common form of systemic vasculitis in adults, preferentially involves large and medium-sized arteries in patients over the age of 50. The classic manifestations are headaches, jaw claudication, polymyalgia rheumatica, and visual symptoms. Acute myeloid leukemia (AML) is a hematopoietic stem cell disorder characterized by a block in the differentiation of hematopoiesis, resulting in the growth of a clonal population of neoplastic cells or blasts. This malignant alteration in hematopoietic stem cells leads to a loss of normal hematopoietic function, which, if left untreated, typically leads to death within weeks to months of its clinical presentation. Although there have been reports of CLL or CML accompanied by several kinds of autoimmune vascular diseases, such as polymyalgia rheumatica, GCA, or necrotizing temporal arteritis, no studies have reported a case of AML with GCA. We experienced an 80-year-old male patient who developed AML 6 years after the diagnosis of GCA. He was under the use of oral glucocorticoid, hydroxychloroquine, and methotrexate at the time of the diagnosis of the AML. This is the first case in Korea to report GCA accompanied by AML.

A New Technique for Inferior Hypogastric Plexus Block: A Coccygeal Transverse Approach: A Case Report

Chronic pelvic pain is a common problem with variable etiology. The sympathetic nervous system plays an important role in the transmission of visceral pain regardless of its etiology. Sympathetic nerve block is effective and safe for treatment of pelvic visceral pain. One of them, the inferior hypogastric plexus, is not easily assessable to blockade by local anesthetics and neurolytic agents. Inferior hypogastric plexus block is not commonly used in chronic pelvic pain patients due to pre-sacral location. Therefore, inferior hypogastric plexus is not readily blocked using paravertebral or transdiscal approaches. There is only one report of inferior hypogastric plexus block via transsacral approach. This approach has several disadvantages. In this case a favorable outcome was obtained by using coccygeal transverse approach of inferior hypogastric plexus. Thus, we report a patient who was successfully given inferior hypogastric plexus block via coccygeal transverse approach to treat chronic pelvic pain conditions involving the lower pelvic viscera.