Poland's syndrome is congenital anomaly which was described first by Alfred Poland in 1841. The clinical features are variable but always include congenital aplasia of fingers and syndactyly. We have experienced a case of Poland's syndrome. This case was male children and revealed thoracic anomaly of right side (absence of pectoral muscles and anterior axillary fold), atrophy of forearm muscles, ipsilateral syndactyly with aplasia of thumb and middle phalanges of 2, 3, 4, 5,th fingers, and ipsilateral congenital radio-ulnar synostosis.
Atrophy ; Child ; Fingers ; Forearm ; Humans ; Male ; Muscles ; Poland ; Syndactyly ; Synostosis ; Thumb

The Ehlers-Danlos syndrome is a familial disorder of connective tissue, and seven distinct clinical forms are reported. Author experienced two cases of the type 3 Ehlers-Danlos syndrome which present hypermobility of the joints, asymmetry of the thorax, anomalies of the thoracic spine, and flatfoot. So, we report these patients with references.
Connective Tissue ; Ehlers-Danlos Syndrome ; Flatfoot ; Humans ; Joints ; Spine ; Thorax

The function of the knee joint is determined by the anatomical integrity of ligamentous structures, however the ligaments and soft tissues which support by the knee joint are too weak to tolerate forceful stresses. The knee joint is one of the most susceptible joints to ligamentous injury. Recently, the incidence of ligamentous injuries of the knee increased because of rise in traffic accidents and participation in sports. For good results, it is very important to make an early diagnosis and to give prompt management. The authors reviewed 67 cases of ligamentous injuries in patients who were admitted and managed at the Department of Orthopaedic Surgery of Presbyterian Medical Center from March of 1976 to December of 1982. The results of the clinical study are as follows: 1. The prevalent age group is the 3rd through 5th decades, and more frequent in males (8. 6 times). 2. The traffic accident is the most common cause of injury (80. 6%) and many ligamentous injuries were associated with fracture (53. 2%). 3. The most common ruptured individual ligament was the medial collateral ligament. The isolated ligamentous rupture of anterior and posterior cruciate ligament was 3 cases each, but most of the cruciate ligaments were combined with other ligament injury. 4. The rupture sites of the individual ligaments were found to be the following: (a) The medial collateral ligament was ruptured most frequently in the mid-substance (45. 2%), with the deep layer ruptured at the femoral attachment and the superficial layer in the mid-substance; (b) The lateral collateral ligament torn at the fibular attachment (62. 5%); (c) The anterior cruciate ligament torn at the femoral and tibial (47. 6%, respectively); (d) The posterior cruciate ligament torn at the femoral attachment (40. 0%). 5. It is significant if the difference between the normal knee and the injured knee on the stress film is more than 10 or 10mm. 6. Excellent and good results were obtained in 95.2% by non-surgical management and in 93.0% by surgical management. 7. Early surgical repairs, made within 2 weeks after injury, gave much better results than late repairs.
Accidents, Traffic ; Anterior Cruciate Ligament ; Clinical Study ; Collateral Ligaments ; Early Diagnosis ; Humans ; Incidence ; Joints ; Knee Joint ; Knee ; Lateral Ligament, Ankle ; Ligaments ; Male ; Posterior Cruciate Ligament ; Protestantism ; Rupture ; Sports

The incidence of diabetes mellitus is in increasing tendency because of expension of medical care, economic development and increasing of the population of the aged person recently. The diabetic foot is one of serious complication of diabetes. With the advancement of the method of treatment of diabetes the other acute metabolic complications are decreased, but the development of diabetic gangrene encounteres at any age of adult life with increasing of incidence according to the duration of diabetes. The most of them requires operation, and its treatment is difficult frequently because of high incidence of wound problem in healing. So it is important to educate and prevent for lowering its incidence. From January 1978 to December 1984, 38 patients with diabetic foot were treated and analysed at Department of Orthopaedic Surgery of Presbyterian Medical Center. The results obtained are as follow. 1. The average of annual incidence of diabetic gangrene was 2.85%. There was increasing tendency of incidence to 1981, thereafter decreasing tendency. 2. 71% of patients with gangrene were in over-50 year age group. 3.72% of patients were in duration of diabetes more than 5 years. 4.79% of patiens were in inadequate treatment before admisson. 5. There was no relationship between fasting blood sugar level and development of gangrence. 6. The most common site of the lesion was big toe. 7. The most common predisposing fector was local pressure (39%), then minor trauma, and burn injury. 8. The primary wound healing rate was 53% after operation. 9. There was no relationship between the lowest palpable artery and primary wound healing. 10. The most common combined complication of diabetes was retinopathy (47%), then neuropathy and hypertension. 11. The rate of bacterial infection was 66%, and the most common organism was staphylococcus aureus (36%).
Adult ; Arteries ; Bacterial Infections ; Blood Glucose ; Burns ; Clinical Study ; Diabetes Mellitus ; Diabetic Foot ; Economic Development ; Fasting ; Gangrene ; Hallux ; Humans ; Hypertension ; Incidence ; Methods ; Protestantism ; Staphylococcus aureus ; Wound Healing ; Wounds and Injuries

Recently, tibial shaft fractures become more severe because of high speed traffic accidents. The open tibial fracture is a difficult one to treat because of its common complications such as infection, soft tissue defect, delayed union and non-union. We studied 37 cases of open tibial fractures which were treated with external fixation. The results are as follows: 1. The average patient age was 29.3 years and male predominated by a ratio of 4.3: l. 2. The traffic accidents were the most common cause of injury. 3. Most cases(67.6%) were type III fracture(by classification of Gustilo and Anderson). 4. Hoffmann external fixator gave rigid fixation and good wound access. 5. There were 28 cases(75.7%) which needed secondary soft tissue reconstruction. 6. The infection rate was 21.5%(8 cases). These all healed with conservative management. There were 5 cases of pin tract infection which healed without developing osteitis. 7. The average union time was 28.7 weeks. In type III fracture there was marked delay in union (average 32.1 weeks). 8. The rate of development of delayed or non-union was 35.1%. In these cases, bony union was obtained with cancellous bone graft.
Accidents, Traffic ; Classification ; External Fixators ; Humans ; Male ; Osteitis ; Soft Tissue Infections ; Tibia ; Tibial Fractures ; Transplants ; Wounds and Injuries

PURPOSE: The gonadotropin-releasing hormone agonist (GnRHa) is widely used to treat patients with precocious puberty. However, its effect on growth is often difficult to predict because of the diverse nature of its causes and presentation. This study aims to show the impact of GnRHa treatment on insulin-like growth factor-1 (IGF-I) and IGF binding protein-3 (IGFBP-3) secretion, growth, and on other parameters that may help estimate the height velocity. METHODS: Data from 60 girls (mean age, 8.8+/-0.7 years) treated with GnRHa were analyzed. Their height, bone age (BA), serum IGF-I, and IGFBP-3 concentrations were measured at the start and after a year of GnRHa treatment. To eliminate the confounding effect of chronological age (CA), the standard deviation scores (SDSs) of their height, IGF-I, and IGFBP-3 concentrations according to their CA at the start and after a year of GnRHa treatment were calculated. We looked for possible correlations between these variables and compared the subgroups based on their height velocities and midparental heights. RESULTS: During their one-year GnRHa therapy, height SDS for CA significantly decreased to 0.81+/-0.83 (P<0.001), but height SDS for BA increased to -0.28+/-0.68 (P<0.001). There was no significant change in serum IGF-I SDS, IGFBP-3 SDS, and IGF-I/IGFBP-3 ratio. The advanced BA was the factor most strongly correlated to the height velocity (R=0.265, P=0.041). CONCLUSION: These findings suggest that GnRHa treatment may affect the height velocity due to mechanisms other than suppression of the IGF-I and IGFBP-3 secretory axis.
Axis ; Body Height* ; Female ; Gonadotropin-Releasing Hormone* ; Humans ; Insulin-Like Growth Factor Binding Protein 3 ; Insulin-Like Growth Factor I ; Puberty, Precocious

Purpose: To describe the clinical outcome of retrobulbar injection of synthetic fillers to correct enophthalmos associated with phthisis bulbi or anophthalmos. Methods: We retrospectively reviewed the medical records of patients who underwent enophthalmos correction using retrobulbar filler injections at Asan Medical Center between January 2015 and October 2019, and who were followed for at least 6 months. We evaluated the number of injections and amount of filler injected, improvement in enophthalmos, interval between injections, and adverse effects of filler injection. Results: The study enrolled five patients (four females and one male). Two patients had anophthalmos after evisceration and three had phthisis bulbi. Two patients received hyaluronic acid (HA) filler only, one had collagen-polymethylmethacrylate (PMMA) filler only, and two had both fillers. The HA and collagen-PMMA filler volumes per injection were 1.0–1.4 and 0.75–1.0 mL, respectively. The average degree of enophthalmos, compared to the contralateral eye, was 1.8 mm; the amount of enophthalmos correction per 1 mL of filler injection was 1.5 mm for HA filler and 1.4 mm for collagen-PMMA filler. The longest duration of enophthalmos correction was 15 months for HA filler and 25 months for collagen-PMMA filler. There were no significant adverse effects, but anterior migration of HA filler was observed in one case that resolved with hyaluronidase injection. Conclusions Retrobulbar filler injection is a safe, effective, minimally invasive procedure for correcting enophthalmos in patients with anophthalmos or phthisis bulbi. If HA filler injection shows good outcomes without adverse effects, semi-permanent fillers can be used for long-term maintenance of enophthalmos correction. Further studies with more patients and long-term follow-up are needed to compare the effectiveness of various fillers.

The drug reaction with eosinophilia and systemic symptom (DRESS) is a severe adverse drug-induced reaction. Acetaminophen is extensively used as an over-the-counter drug as well as a medical therapeutic. In spite of its frequent use, drug eruptions related to DRESS caused by acetaminophen are rare. This case report describes a 9-year-old boy who experienced fever and maculopapular skin rashes after taking acetaminophen. Leukocytosis with marked eosinophilia and slightly elevated levels of liver enzymes were observed. Symptoms resolved after withdrawal of acetaminophen, followed by intravenous methylprednisolone administration. The diagnosis of DRESS induced by acetaminophen was confirmed by an oral challenge test after 2 months. No allergic reactions to ibuprofen were observed.
Acetaminophen ; Child* ; Diagnosis ; Drug Eruptions ; Drug Hypersensitivity ; Eosinophilia* ; Exanthema ; Fever ; Humans ; Hypersensitivity ; Ibuprofen ; Leukocytosis ; Liver ; Male* ; Methylprednisolone

G protein-coupled receptors (GPCRs) are membrane receptors whose agonist-induced dynamic conformational changes trigger heterotrimeric G protein activation, followed by GRK-mediated phosphorylation and arrestin-mediated desensitization. Cytosolic regions of GPCRs have been studied extensively because they are direct contact sites with G proteins, GRKs, and arrestins. Among various cytosolic regions, the role of helix 8 is least understood, although a few studies have suggested that it is involved in G protein activation, receptor localization, and/or internalization. In the present study, we investigated the role of helix 8 in dopamine receptor signaling focusing on dopamine D1 receptor (D1R) and dopamine D2 receptor (D2R). D1R couples exclusively to Gs, whereas D2R couples exclusively to Gi. Bioinformatic analysis implied that the sequences of helix 8 may affect GPCR-G protein coupling selectivity; therefore, we evaluated if swapping helix 8 between D1R and D2R changed G protein selectivity. Our results suggest that helix 8 is not involved in D1R-Gs or D2R-Gi coupling selectivity. Instead, we observed that D1R with D2R helix 8 or D1R with an increased number of hydrophobic residues in helix 8 relative to wild-type showed diminished β-arrestin-mediated desensitization, resulting in increased Gs signaling.
Arrestin ; Arrestins ; Computational Biology ; Cytosol ; Dopamine ; Family Characteristics ; GTP-Binding Proteins ; Membranes ; Phosphorylation ; Receptors, Dopamine D1 ; Receptors, Dopamine D2 ; Receptors, Dopamine

Purpose: Early nutritional support (ENS) for critically ill patients is promoted by many studies. However, there is a lack of data evaluating its necessity in general wards. This study aims to determine the impact of ENS on patients in general wards. Methods: Patients aged 18 and above, admitted to the Jeonbuk National University Hospital in Jeonju from January 2020 to December 2020, who were eligible for nutritional support and hospitalized for at least 7 days were included in the study. We divided the patients into two groups: the ENS group, who received nutritional support within 48 hours of admission, and the control group, who received it after 48 hours. Results: Among 1,077 patients, 146 met the inclusion criteria. The ENS group (n=38) and the control group (n=108) were compared retrospectively. There was a significant age difference between the two groups (P=0.028). The admission ratio to the intensive care unit (ICU) in the ENS group was significantly lower than that in the control group (10.2% vs.26.3%, P=0.019). The calorie support rate (%) and protein support rate (%) in the ENS group were significantly higher than in the control group (50.12%±23.30% vs. 38.56%±18.02%, P=0.006; 44.61%±25.07% vs. 32.07%±22.76%, P=0.002, respectively). After propensity score matching, the ENS was significantly associated with ICU low admissions (odds ratio 0.08, 95% confidence interval 0.01–0.69, P=0.022). Conclusion A future multi-center study considering underlying diseases is needed to provide additional scientific evidence to support the effects of ENS.