Nasoalveolar molding (NAM) device is an effective treatment for protruding maxilla in infants with cleft palate. However, only a few studies have investigated the effect of NAM devices on the treatment of protruding maxilla in infants with cleft lip only. We have designed a combination treatment using NAM devices prior to cheiloplasy for cleft lip-only patients with severe anterior protrusion of the premaxilla. Three cleft lip-only infants with 1-cm or more of premaxilla protrusion were included. Definitive cheiloplasty was performed at 6 months of age without any preoperative correction in infant 1. Cheiloplasty was performed in conjunction with the use of NAM device and lip adhesion in infants 2 and 3. Postoperative columella length and anterior-posterior dimension of the protruding premaxilla were compared amongst the infants. We were able to obtain satisfactory postoperative columella length and general nasal appearance.
Cleft Lip* ; Cleft Palate ; Fungi* ; Humans ; Infant ; Lip* ; Maxilla ; Orthodontic Appliances

A case of an anterior communicating(Acom) artery aneurysm in a 36-year-old woman with polycystic kidney, and a case of multiple aneurysms(Acom. And anterior choroidal artery aneurysm) in a 49-year-old man with Marfan's syndrome are reported. Polycystic kidney was confirmed by abdominal ultrasonography and computed tomography. Marfan's syndrome was diagnosed with skeletal abnormalities(dolichocephaly, highly arched palate, cleft palate, arachnodactyly and tall slender height with excessive length of limbs) and marked elongation and tortuosity of the vertebral artery on cerebral angiography. Those patients were treated with the clipping of aneurysms. We will discuss the pathogenesis of cerebral aneurysm in those diseases with stress on the important points of management.
Adult ; Aneurysm ; Arachnodactyly ; Arteries ; Cerebral Angiography ; Choroid ; Cleft Palate ; Female ; Humans ; Intracranial Aneurysm* ; Marfan Syndrome* ; Middle Aged ; Palate ; Polycystic Kidney Diseases* ; Ultrasonography ; Vertebral Artery

Congenital myotonic dystrophy type 1 (DM1) presents severe generalized weakness, hypotonia, and respiratory compromise after delivery with high mortality and poor prognosis. We presented a congenital DM1 of premature twins in the 30th week of gestation. These twins were conceived by in vitro fertilization (IVF). Both babies presented apnea and hypotonia and had characteristic facial appearance. They were diagnosed DM1 by genetic method. They were complicated by chylothorax and expired at 100 and 215 days of age, respectively. Mother was diagnosed DM1 during the evaluation of babies. This is the first report on congenital DM1 which accompanied the chylothorax. More investigation on the association with chylothorax and congenital DM1 is recommended. With a case of severe neonatal hypotonia, congenital DM1 should be differentiated in any gestational age. Finally, since DM1 is a cause of infertility, we should consider DM1 in infertility clinic with detailed history and physical examination.
Adult ; Apnea/etiology ; Blotting, Southern ; Chylothorax/complications ; Female ; Fertilization in Vitro ; Humans ; Infant, Newborn ; Infant, Premature ; Microsatellite Repeats/genetics ; Muscle Hypotonia/etiology ; Myotonic Dystrophy/complications/*diagnosis/radiography ; Twins

PURPOSE: To evaluate the effect of oral sildenafil therapy in neonates with persistent pulmonary hypertension of the newborn (PPHN) METHODS: We conducted a retrospective review of 32 neonates > or =35 weeks' gestation and fraction of inspired oxygen (FiO2) 1.0 with PPHN. The first dose (0.5 mg/kg) of oral sildenafil was started and 1 mg/kg was given every 6 hour thereafter. Mean airway pressure (MAP), FiO2, oxygenation index (OI), mean arterial blood pressure (MBP) were documented before and 6, 12, 24, and 48 hours after sildenafil. For adverse effects, gastrointestinal symptoms, brain ultrasound, funduscopy and auditory brainstem response results were evaluated. RESULTS: The underlying diseases of PPHN (n=32) were meconium aspiration syndrome (n=9), respiratory distress syndrome (n=8), pneumonia (n=3), and idiopathic (n=12). Thirty-one neonates survived; 3 neonates were transferred for inhaled nitric oxide (iNO) and all of them survived. In 28 infants, FiO2 and OI improved significantly by 6 hours and MAP improved significantly by 48 hours after initiation of sildenafil. There were no clinically significant adverse effects of sildenafil. CONCLUSION: Sildenafil may be an effective and safe agent for near-term and term neonates with PPHN, providing significant improvement in oxygenation, and thus may be especially useful in the treatment of PPHN in hospitals without iNO.
Arterial Pressure ; Brain ; Evoked Potentials, Auditory, Brain Stem ; Humans ; Hypertension, Pulmonary ; Infant ; Infant, Newborn ; Meconium Aspiration Syndrome ; Nitric Oxide ; Oxygen ; Piperazines ; Pneumonia ; Pregnancy ; Purines ; Retrospective Studies ; Sulfones ; Sildenafil Citrate

PURPOSE: A limited number of studies have examined the link between F-wave abnormalities and clinical presentation in pediatric Guillain-Barré syndrome (GBS). Therefore, this study examined the importance of F-wave abnormalities as a prognostic factor in pediatric GBS patients. METHODS: The records and electrodiagnostic studies (EDS) of 70 GBS patients were retrospectively evaluated, and divided into 2 groups according to the results of EDS. Group A (n=33) presented with F-wave abnormalities, and group B (n=26) exhibited normal findings. We compared laboratory reports, clinical features, response to treatment, and prognosis between the 2 groups. RESULTS: Motor weakness was the most frequently observed symptom for either group. Clinically, the incidence of fever and upper respiratory symptoms differed between the 2 groups, while the prevalence of abnormal deep tendon reflex (DTR) was significantly higher in group A than B (P<0.05). Patients diagnosed with GBS had received intravenous immunoglobulin treatment: 94% in group A and 58% in group B. Furthermore, significantly greater numbers of patients in group A showed H-reflex abnormalities and poor prognosis compared with group B (P<0.05). CONCLUSION: This study demonstrated that F-waves are a clinically important prognostic factor in GBS. F-wave abnormalities were associated with abnormal DTR and poor prognosis in patients. Limited studies have examined the link between F-wave abnormalities and clinical results; therefore, further randomized controlled studies are needed to confirm the clinical characteristics and efficacy of treatments.
Child* ; Fever ; Guillain-Barre Syndrome* ; H-Reflex ; Humans ; Immunoglobulins ; Incidence ; Prevalence ; Prognosis ; Reflex, Abnormal ; Retrospective Studies

PURPOSE: We describe a 54-year-old woman with systemic lupus erythematosus (SLE) who suddenly presented with chorea and had positive antiphospholipid antibodies. F-18 FDG PET showed abnormally increased glucose metabolism in bilateral putamen and primary motor cotex. Tc-99m ECD SPECT also showed abnormally increased regional cerebral blood flow in bilateral putamen. She was treated with corticosteroid and aspirin after which the symptoms improved. Four months later, follow up F-18 FDG PET showed improvement with resolution of hypermetabolism in bilateral putamen. This case suggests that striatal hypermetabolism is associated with chorea in SLE.
Antibodies, Antiphospholipid ; Aspirin ; Chorea* ; Female ; Follow-Up Studies ; Glucose ; Humans ; Lupus Erythematosus, Systemic* ; Metabolism ; Middle Aged ; Putamen ; Tomography, Emission-Computed, Single-Photon

Central catheterization has been widely employed because of its various uses. However, there are many complications which are classified as mechanical complications, infectious complications, and thrombotic complications. Especially, subclavian catheterization has a high rate of mechanical complication such as pneumothrax and hemothorax. We experienced an unusual complication of central catheter misplacement in thoracic surgery and identified it with video assisted thoracoscopic equipment. We consider hemothorax to be the cause of this type of central catheter misplacement.
Catheterization ; Catheterization, Central Venous ; Catheters ; Central Venous Catheters ; Hemothorax ; Thoracic Surgery ; Thoracic Surgery, Video-Assisted

PURPOSE: In patients with IgA nephropathy, more than 1 g/day of proteinuria is a risk factor to develop end-stage renal failure. Uncommonly, patients with IgA nephropathy exhibit negative proteinuria on routine dipstick test during follow-up examination, spontaneously or after use of ACEI or ARB. We evaluated whether no proteinuric patients have good prognosis or not. METHODS: 41 patients who had no proteinuria on routine urinalysis for more than 6 months, were classified into spontaneous remission group (25 cases, SR) without any treatment and drug-induced remission group (16 cases, DR) with treatment of ACEI/ARB or both, were analyzed for clinical findings and renal function, retrospectively. We examined spot urine protein/creatinine ratio (Up/c) to evaluate exact amount of proteinuria and GFR was estimated by MDRD equation. RESULTS: Twenty eight percent of cases in SR and 50 percent in DR showed spot Up/c 0.3-1 g/g. After follow-up of 58+/-41 (9-192) months in SR and 79+/-56 (35-192) months in DR, the stages of CKD shifted to advanced levels as follows; in SR group, 11, 10, 4 patients in stage 1 (GFR> or =90 ml/min/ 1.73m2), 2 (GFR 60-89 ml/min/1.73m2), 3 (GFR 30-59 ml/min/1.73m2) to 7, 13, 5 patients, respectively; in DR, 7, 8, 1 patients in stage 1, 2, 3 to 3, 8, 5 patients, respectively. There was a tendency of slow decreasing GFR in both groups but no case progressed to CKD stage 4 and 5. CONCLUSION: Of IgA nephropathy patients with no proteinuria on routine urinalysis, 30-50% of patients have proteinuria 0.3-1.0 g/g on spot Up/c and there was also a risk of progression.
Follow-Up Studies ; Glomerulonephritis, IGA ; Humans ; Immunoglobulin A ; Kidney Failure, Chronic ; Prognosis ; Proteinuria ; Remission, Spontaneous ; Retrospective Studies ; Risk Factors ; Urinalysis

Elevated lactate levels are associated with acute illnesses, and the mortality is high. Here, we report a case of lactate-containing peritoneal dialysis (PD) solution inducing lactic acidosis corrected by changing to hemodialysis (HD). This 70-year-old female patient was treated with PD 8 months previously for end-stage renal disease caused by diabetes mellitus. She was admitted complaining of general weakness. Initial lactate level was 22.1 mg/dL and increased to 62.4 mg/dL showing high anion gap metabolic acidosis and compensatory hyperventilation. There are no definite causes of lactic acidosis besides the use of PD solutions containing a lactate component. The patient's lactate level was decreased after temporarily changing the dialysis modality to HD. Her lactate level was increased again after restarting PD, and decreased to normal after restarting HD. We report this case because physicians should consider lactate-containing PD solution as a possible cause of lactic acidosis.
Acid-Base Equilibrium ; Acidosis ; Acidosis, Lactic* ; Aged ; Diabetes Mellitus ; Dialysis ; Female ; Humans ; Hyperventilation ; Kidney Failure, Chronic ; Lactic Acid* ; Mortality ; Peritoneal Dialysis* ; Renal Dialysis*

Endoscopic thyroidectomy is gaining popularity, but it can increase the risk of certain complications. Carbon dioxide insufflation in the neck may cause adverse effects on hemodynamic and ventilatory aspects. We report the anesthetic course and complications that were encountered during endoscopic thyroidectomy. Although the surgery was successful, the patient developed signs of hypercarbia, subcutaneous emphysema and pneumothorax.
Carbon Dioxide ; Hemodynamics ; Humans ; Insufflation ; Neck ; Pneumothorax ; Subcutaneous Emphysema ; Thyroidectomy