The usual form of neurofibromatosis is characterized by cafe-au-Iait skin pigmentation and pedunculated fibrous skin tumors but in the severe form, malformations and tumors of the central nervous system may be associated with von Recklinghausen's disease. Schwannoma of the spinal sensory roots, of the vestibular part of 8th cranial nerve, and of the sensory root of the 5th cranial nerve are particularly common. Outside the central nervous system, other neuroectodermal tumors may develop including renal, pancreas, and suprarenal tumors. Recently, neurofibromatosis is subdivided into type 1 and 2, according to the site of involvement. Type 1 is classic von Recklinghausen's disease, and type 2 represents bilateral acoustic neurinomas. The authors experienced 2 cases neurofibromatosis type 1 and 1 case of neurofibromatosis type 2 in which neurofibromas involve both cerebello-pontine angles, multiple levels of spinal cord and cauda equina.
Cauda Equina ; Central Nervous System ; Cranial Nerves ; Neurilemmoma ; Neuroectodermal Tumors ; Neurofibroma ; Neurofibromatoses* ; Neurofibromatosis 1 ; Neurofibromatosis 2 ; Pancreas ; Skin ; Skin Pigmentation ; Spinal Cord

OBJECTIVE: Postoperative delirium (POD) is characterized by an acute change in cognitive function and can result in longer hospital stays, higher morbidity rates, and more frequent discharges to long-term care facilities. In this study, we investigated the incidence and risk factors of POD in 224 patients older than 70 years of age, who had undergone a neurosurgical operation in the last two years. METHODS: Data related to preoperative factors (male gender, >70 years, previous dementia or delirium, alcohol abuse, serum levels of sodium, potassium and glucose, and co morbidities), perioperative factors (type of surgery and anesthesia, and duration of surgery) and postoperative data (length of stay in recovery room, severity of pain and use of opioid analgesics) were retrospectively collected and statistically analyzed. RESULTS: POD appeared in 48 patients (21.4%) by postoperative day 3. When we excluded 26 patients with previous dementia or delirium, 17 spontaneously recovered by postoperative day 14, while 5 patients recovered by postoperative 2 months with medication, among 22 patients with newly developed POD. The univariate risk factors for POD included previously dementic or delirious patients, abnormal preoperative serum glucose level, pre-existent diabetes, the use of local anesthesia for the operation, longer operation time (>3.2 hr) or recovery room stay (>90 min), and severe pain (VAS>6.8) requiring opioid treatment (p<0.05). Backward regression analysis revealed that previously dementic patients with diabetes, the operation being performed under local anesthesia, and severe postoperative pain treated with opioids were independent risk factors for POD. CONCLUSION: Our study shows that control of blood glucose levels and management of pain during local anesthesia and in the immediate postoperative period can reduce unexpected POD and help preventing unexpected medicolegal problems and economic burdens.
Alcoholism ; Analgesics, Opioid ; Anesthesia ; Anesthesia, Local ; Blood Glucose ; Delirium ; Dementia ; Glucose ; Humans ; Incidence ; Length of Stay ; Long-Term Care ; Pain, Postoperative ; Postoperative Period ; Potassium ; Recovery Room ; Retrospective Studies ; Risk Factors ; Sodium

PURPOSE: To report the clinical results from surgical treatment for clavicle shaft fracture by percutaneous intramedullary fixation with Steinmann pins. MATERIALS AND METHODS: Between January 2004 and June 2014, the medical records of 135 patients who underwent percutaneous intramedullary fixation with Steinmann pins were reviewed. The mean follow-up periods were 15 months. The functional results were evaluated with The Disabilities of the Arm, Shoulder and Hand (DASH) score and Constant score. The clinical results were evaluated with the shortened length of the clavicle, length of surgical wound, operation time and Kang's criteria. RESULTS: The mean bone union period was 11.6 weeks (8–16 weeks). The mean DASH score was 11.8. The mean Constant score was 91.2. The mean shortened length of the clavicle was less than 20 mm. The mean length of surgical wound was 1.2 cm (0.7–1.5 cm). The mean operation time was 18 minutes (10–35 minutes). Using Kang's criteria, 131 out of 135 patients (97.0%) showed good results. Complications included were 3 pin migrations and 2 non-unions. CONCLUSION: Percutaneous intramedullary fixation with Steinmann pins showed good results for treating clavicle shaft fracture.
Arm ; Clavicle* ; Follow-Up Studies ; Hand ; Humans ; Medical Records ; Shoulder ; Wounds and Injuries

Endometriosis generally occurs in the pelvis, particularly in the broad ligament, round ligament, ovaries, fallopian tubes or uterosacral ligament. However, many unusual sites have been described, including the umbilicus, cesarean section scars, hernia sacs, appendix, vagina, vulva, omentum and perineum. We experienced a case of perineal endometriosis diagnosed according to cyclic perineal pain associated with menstration and report it with brief review of literature.
Appendix ; Broad Ligament ; Cesarean Section ; Cicatrix* ; Endometriosis* ; Episiotomy* ; Fallopian Tubes ; Female ; Hernia ; Ligaments ; Omentum ; Ovary ; Pelvis ; Perineum ; Pregnancy ; Round Ligament of Uterus ; Umbilicus ; Vagina ; Vulva

Unlike most secretory cells, high extra cellular calcium inhibits rather than stimulates hormonal secretion in several cells such as parathyroid cells, Juxtaglomerular cells and osteoclast. To gain further insight into the common but unique stimulus-secretion coupling mechanism in these cells, bovine parathyroid slices were incubated in various conditions of Krebs-Ringer (KR) solution containing essential amino acids. Parathyroid cells showed the inverse dependency of secretion on extra cellular calcium concentration as we expected. Ammonium acetate overcame the inhibitory effect of 2.5 mM of calcium and the maximum effect was as much as the five times of the basal value, while there was a little additive effect under 0 mM CaCl2. PTH secretion was biphasic according to the change of extra cellular osmolarity and the lowest response was observed at 300 mOsm/l. In Na-rich KR solution, high concentration of nigericin (> 10(-4)M) completely overcame the inhibitory effect of 2.5 mM CaCl2 and the maximum stimulatory effect was 8 times greater whereas it was only 2 times greater without CaCl2. In K-rich KR solution that abolished the K-gradient between the extra cellular solution and the cytoplasm, the rate of PTH secretion increased, and furthermore the addition of nigericin increased the rate of secretion significantly. The results above suggested that the osmotic swelling of the secretory vesicle in parathyroid cells might promote exocytosis as in Juxtaglomerular cells. We propose that the swelling of the vesicle is also prerequisite for secretion in several cells inhibited paradoxically by Ca++, whatever the signal transduction pathway for swelling of the secretory granules induced by the lowering of Ca++ in cytoplasm are.
Acetates/pharmacology ; Animal ; Body Fluids/*metabolism ; Cattle ; Cell Membrane Permeability ; Ionophores/pharmacology ; Mannitol/pharmacology ; Nigericin/pharmacology ; Osmosis ; Parathyroid Glands/drug effects/*secretion ; Parathyroid Hormones/*secretion ; Support, Non-U.S. Gov't

PURPOSE: For certain forms of childhood epilepsy that remain uncontrolled despite adequate treatment with standard antiepileptic medication, surgical therapy should be considered as a potential treatment. The prognosis for seizure control after early surgery is favorable and is at least comparable with that of adults. With the exception of the obvious benefit conferred by alleviating seizures at a younger age, early surgery also later improves psychosocial status and adaptive function. This study was performed to evaluate the efficacy of epilepsy surgery. METHODS: We analyzed the results of 28 cases of intractable childhood epilepsy who underwent epilepsy surgery at the epilepsy center of Dongsan Medical Center between February, 1993 and January, 1996. They followed up for at least 15 months after surgery. Seizures began at 14 days to 15 years (mean 6.3 years) after birth and had been refractory to antiepileptic medications. Presurgical evaluations of epilepsy included detailed clinical history, scalp/sphenoidal EEG, Video-EEG monitoring, neuroimaging, neuropsychological test, Wada test and invasive study with subdural electrodes. RESULTS: Temporal lobectomy (with or without corticectomy) was performed in 13 cases, extratemporal lobectomy in 11 cases (frontal lobe n=7, parietal lobe n=2, frontoparietal n=1, parietooccipital n=1), functional hemispherectomy in two cases and corpus callosotomy in two cases. The surgical outcome was better in temporal lobe epilepsy compared with that of extratemporal lobe epilepsy. In temporal lobe epilepsy, seven of 13 cases had class I outcome grade, four cases had class II and the rest had class III and class IV. In extratemporal lobe epilepsy, five of 11 cases had class I outcome and the remainders had class III, IV. CONCLUSION: Our results agree with previous reports that epilepsy surgery can provide relief from intractable seizure in pediatric patients, but more extensive study for the patients' cognitive and behavior status will be necessary.
Adult ; Electrodes ; Electroencephalography ; Epilepsy* ; Epilepsy, Temporal Lobe ; Hemispherectomy ; Humans ; Neuroimaging ; Neuropsychological Tests ; Parietal Lobe ; Parturition ; Prognosis ; Seizures

BACKGROUND: Making the diagnosis of the follicular variant of papillary thyroid carcinoma (FVPTC) is often difficult, and there are no accurate immunohistochemical or molecular markers. The purpose of this study is to evaluate performing immunohistochemistry to make the diagnosis of FVPTC. METHODS: A total of 249 thyroid lesions were studied. We made the tissue microarray, and we assessed the expression of HBME-1, galectin-3, CD56, and p63. RESULTS: Galectin-3, HBME-1, and p63 were positive in 79.7%, 79.7%, and 15.9% of the FVPTC, respectively. These immunohistochemical features of FVPTC were between those of classic papillary thyroid carcinoma (CPTC) and those of non-PTC. The CD56 expression was positive in 75.4% of the FVPTC, which is much higher than that of the CPTC (28.3%), and even higher than that of the non-PTC lesions (60%). Comparing FVPTC with CPTC, the expression of galectin-3 was significantly higher and the expression of CD56 was significantly lower in the CPTCs. Comparing the FVPTC with follicular carcinoma (FC), the expression of all the markers was significantly higher in the FVPTC. Comparing PTC with FC, the expression of CD56 was lower and the expressions of the other markers were higher in the PTCs. CONCLUSIONS: Galectin-3, HBME-1, and p63 can help make the diagnosis of FVPTC, and a cocktail of these markers can be even more useful. But CD56 is not thought to be useful to make the diagnosis of FVPTC.
Carcinoma ; Carcinoma, Papillary ; Factor IX ; Galectin 3 ; Immunohistochemistry ; Thyroid Gland ; Thyroid Neoplasms

Congenital myotonic dystrophy is a severe and early-onset form of myotonic dystrophy (DM) with a prevalence of 2.5-5.5/100,000 live births. Expansion of the trinucleotide CTG repeat in the 3 untranslated region of the DM gene, which is located at a chromosome 19q13.3 is a common mutation in DM. Clinical features are generalized hypotonia (floppy infant), respiratory and feeding difficulty, and the neonatal mortality rate is approximately 40%. We experienced a case of recurrent congenital myotonic dystrophy, and report with a review of related literatures. Women with recurrent neonatal hypotonia or ultrasonographic evidence of hypotonia, including positional abnormalities of the extremities and idiopathic polyhydramnios, should be offered testing for the genetic studies for myotonic mutation, such as PCR (Polymerase chain reaction) analysis and Southern blot analysis.
Blotting, Southern ; Diagnosis* ; Extremities ; Female ; Humans ; Infant ; Infant Mortality ; Live Birth ; Muscle Hypotonia ; Myotonic Dystrophy* ; Polyhydramnios ; Polymerase Chain Reaction ; Prenatal Diagnosis ; Prevalence ; Untranslated Regions

Congenital myotonic dystrophy is a severe and early-onset form of myotonic dystrophy (DM) with a prevalence of 2.5-5.5/100,000 live births. Expansion of the trinucleotide CTG repeat in the 3 untranslated region of the DM gene, which is located at a chromosome 19q13.3 is a common mutation in DM. Clinical features are generalized hypotonia (floppy infant), respiratory and feeding difficulty, and the neonatal mortality rate is approximately 40%. We experienced a case of recurrent congenital myotonic dystrophy, and report with a review of related literatures. Women with recurrent neonatal hypotonia or ultrasonographic evidence of hypotonia, including positional abnormalities of the extremities and idiopathic polyhydramnios, should be offered testing for the genetic studies for myotonic mutation, such as PCR (Polymerase chain reaction) analysis and Southern blot analysis.
Blotting, Southern ; Diagnosis* ; Extremities ; Female ; Humans ; Infant ; Infant Mortality ; Live Birth ; Muscle Hypotonia ; Myotonic Dystrophy* ; Polyhydramnios ; Polymerase Chain Reaction ; Prenatal Diagnosis ; Prevalence ; Untranslated Regions

BACKGROUND/AIMS: We sought to identify predictors of significant tricuspid regurgitation (TR) after successful permanent pacemaker (PPM) implantation in Korean patients. METHODS: Of 404 patients who underwent PPM implantation, 187 patients who had both baseline and follow-up echocardiographic examinations were assigned to one of two groups: no development or change in TR (Group I, n = 172, 65.5 +/- 13.7 years) versus the development of significant TR (Group II, n = 15, 72.1 +/- 8.3 years). Clinical, laboratory, and echocardiographic variables were compared between the two groups. RESULTS: Overall, the grade of TR was significantly aggravated from 0.46 +/- 0.73 to 0.81 +/- 0.84 (p < 0.001) during 3.1 +/- 1.8 years of follow-up (0.49 +/- 0.75 to 0.69 +/- 0.74 in Group I, p < 0.001; 0.13 +/- 0.35 to 2.27 +/- 0.46 in Group II, p < 0.001). The de novo development or aggravation of TR was observed in 66 patients (35.3%), and significant TR developed in 15 patients (8.0%). The presence of atrial fibrillation (AF) was significantly higher (53.3 vs. 18.6%, p = 0.002), and the implantation of a ventricle pacing, ventricle sensing, inhibited by ventricular event (VVI) type pacemaker was more frequent in Group II than in Group I (46.7 vs. 15.1%, p = 0.002). Other variables were not different between the groups. CONCLUSIONS: The development or aggravation of TR was not rare after successful PPM implantation, even though the development of significant TR was uncommon. The presence of AF and the implantation of a VVI type pacemaker were predictors of the development of significant TR. Together, the results of this study suggest that the development or aggravation of TR should be monitored carefully after PPM implantation.
Atrial Fibrillation ; Echocardiography ; Follow-Up Studies ; Humans ; Tricuspid Valve Insufficiency*