Evaluating Novel SP-B and SP-C Synthetic Analogues for Pulmonary Surfactant Efficacy.

Pulmonary surfactants, a complex assembly of phospholipids and surfactant proteins such as SP-B and SP-C, are critical for maintaining respiratory system functionality by lowering surface tension (ST) and preventing alveolar collapse. Our study introduced five synthetic SP-B peptides and one SP-C peptide, leading to the synthesis of CHAsurf candidates (CHAsurf-1 to CHAsurf-5) for evaluation. We utilized a modified Wilhelmy balance test to assess the surface tension properties of the surfactants, measuring spreading rate, surface adsorption, and ST-area diagrams to comprehensively evaluate their performance. Animal experiments were performed on New Zealand white rabbits to test the efficacy of CHAsurf-4B, a variant chosen for its economic viability and promising ST reduction properties, comparable to Curosurf®. The study confirmed that higher doses of SP-B in CHAsurf-4 are associated with improved ST reduction. However, due to cost constraints, CHAsurf-4B was selected for in vivo assessment. The animal model revealed that CHAsurf-4B could restore alveolar structure and improve lung elasticity, akin to Curosurf®. Our research highlights the significance of cysteine residues and disulfide bonds in the structural integrity and function of synthetic SP-B analogues, offering a foundation for future surfactant therapy in respiratory disorders. This study's findings support the potential of CHAsurf-4B as a therapeutic agent, meriting further investigation to solidify its role in clinical applications.

Nationwide Long-Term Growth and Developmental Outcomes of Infants for Congenital Anomalies in the Digestive System and Abdominal Wall Defects With Surgery in Korea.

BACKGROUND: Infants with congenital anomalies of the digestive system and abdominal wall defects requiring surgery are at risk of growth and developmental delays. The aim of this study was to analyze long-term growth and developmental outcomes for infants with congenital anomalies of the digestive system and abdominal wall defects who underwent surgery in Korea. METHODS: We extracted data from the Korean National Health Insurance Service database for the years 2013-2019. Major congenital anomalies were defined according to the International Classification of Diseases-10 and surgery insurance claim codes. The χ² test and the Cochran-Armitage trend test were performed for data analysis. RESULTS: A total of 4,574 infants with major congenital anomalies in the digestive system and abodminal wall defects, who had undergone surgey, were reviewed. Anorectal obstruction/stenosis was the most prevalent anomaly (4.9 per 10,000 live births). The prevalence of congenital anomalies of the digestive system was 15.5 per 10,000 live births, and that of abdominal wall defects was 1.5 per 10,000 live births. Seven percent of infants with congenital anomalies in the digestive system died, of which those with diaphragmatic hernia had the highest mortality rate (18.8%). Among 12,336 examinations at 6, 12, 24, 36, 48, 60, and 72 months of age, 16.7% showed a weight below the 10th percentile, 15.8% had a height below the 10th percentile, and 13.2% had a head circumference below the 10th percentile. Abnormal developmental screening results were observed in 23.0% of infants. Infants with esophageal atresia with/without tracheoesophageal fistula most often had poor growth and development. Delayed development and cerebral palsy were observed in 490 (10.7%) and 130 (2.8%) infants respectively. Comparing the results of infants born in 2013 between their 24- and 72-month health examinations, the proportions of infants with poor height and head circumference growth increased by 6.5% and 5.3%, respectively, whereas those with poor weight growth and abnormal developmental results did not markedly change between the two examinations. CONCLUSION: Infants with congenital anomalies of the digestive system and abdominal wall defects exhibit poor growth and developmental outcomes until 72 months of age. Close monitoring and careful consideration of their growth and development after discharge are required.

Major Congenital Anomalies in Korean Livebirths in 2013-2014: Based on the National Health Insurance Database.

BACKGROUND: In Korea, there have been no reports comparing the prevalence of major congenital anomalies with other countries and no reports on surgical treatment and long-term mortality. We investigated the prevalence of 67 major congenital anomalies in Korea and compared the prevalence with that of the European network of population-based registries for the epidemiological surveillance of congenital anomalies (EUROCAT). We also investigated the mortality and age at death, the proportion of preterm births, and the surgical rate for the 67 major congenital anomalies. METHODS: Korean National Health Insurance claim data were obtained for neonates born in 2013-2014 and admitted within one-year-old. Sixty-seven major congenital anomalies were defined by medical diagnoses classified by International Classification of Diseases-10 codes according to the EUROCAT definition version 2014. Mortality and surgery were defined if any death or surgery claim code was confirmed until 2020. Poisson distribution was used to calculate the 95% confidence interval of the congenital anomaly prevalence. RESULTS: The total prevalence of the 67 major anomalies was 433.5/10,000 livebirths. When compared with the prevalence of each major anomaly in EUROCAT, the prevalence of spina bifida, atrial septal defect (ASD), congenital megacolon, hip dislocation and/or dysplasia and skeletal dysplasia were more than five times higher in Korea. In contrast, the prevalence of aortic atresia/interrupted aortic arch and gastroschisis was less than one-fifth in Korea. The proportion of preterm births was 15.7%; however, more than 40% of infants with anencephaly, annular pancreas and gastroschisis were preterm infants. Additionally, 29.2% of the major anomalies were admitted to the neonatal intensive care units at birth, and 25.6% received surgical operation. The mortality rate was 1.7%, and 78.2% of the deaths occurred within the first year of life. However, in neonates with tricuspid valve atresia and stenosis, duodenal atresia or stenosis, and diaphragmatic hernia, more than half died within their first month of life. ASD and ventricular septal defect were the most common anomalies, and trisomy 18 and hypoplastic left heart syndrome were the most fatal anomalies. All infants with aortic atresia/interrupted aortic arch and conjoined twins received surgery. CONCLUSION: The proportion of surgeries, preterm births and mortality was high in infants with major congenital anomalies. The establishment of a national registry of congenital anomalies and systematic support by national medical policies are needed for infants with major congenital anomalies in Korea.

Effects of depth of neuromuscular blockade on the BIS-guided propofol requirement: A randomized controlled trial.

BACKGROUND: Deep neuromuscular blockade is considered beneficial for improving the surgical space condition during laparoscopic surgery. Adequacy of the surgical space condition may affect the anesthetists' decision regarding titration of depth of anesthesia. We investigated whether deep neuromuscular blockade reduces the propofol requirement under bispectral index monitoring compared to moderate neuromuscular blockade. METHODS: Adult patients undergoing elective laparoscopic colorectal surgery were randomly allocated to a moderate or deep group. A train-of-four count of 1-2 in the moderate group, and a post-tetanic count of 1-2 in the deep group, were maintained by continuous infusion of rocuronium. The induction and maintenance of anesthesia were achieved by target-controlled infusion of propofol and remifentanil. The dose of propofol was adjusted to maintain the bispectral index in the range of 40-50. The remifentanil dose was titrated to maintain the systolic blood pressure to within 20% of the ward values. RESULTS: A total of 82 patients were included in the analyses. The mean±SD dose of propofol was 7.54 ±â€Š1.66 and 7.42 ±â€Š1.01 mg·kg-1·h-1 in the moderate and deep groups, respectively (P = .104). The mean±SD dose of remifentanil was 4.84 ±â€Š1.74 and 4.79 ±â€Š1.77 µg kg-1 h-1 in the moderate and deep groups, respectively (P = .688). In comparison to the moderate group, the deep group showed significantly lower rates of intraoperative patient movement (42.9% vs 22.5%, respectively, P = .050) and additional neuromuscular blocking agent administration (76% vs 53%, respectively, P = .007). Postoperative complications, including pulmonary complications, wound problems and reoperation, were not different between the two groups. CONCLUSION: Deep neuromuscular blockade did not reduce the bispectral index-guided propofol requirement compared to moderate neuromuscular blockade during laparoscopic colon surgery, despite reducing movement of the patient and the requirement for a rescue neuromuscular blocking agent. TRIAL REGISTRATION: Clinicaltrials.gov (NCT03890406).

Congenital Anomalies in Very-Low-Birth-Weight Infants: A Nationwide Cohort Study.

BACKGROUND: Management of newborn infants with congenital anomalies is challenging and requires a multidisciplinary approach. The prevalence of congenital anomalies in very-low-birth-weight infants (VLBWIs; birth weight <1,500 g) has been rarely reported. OBJECTIVES: The aim of this study was to investigate the epidemiology of congenital anomalies in VLBWIs and the association with early mortality and major morbidities. STUDY DESIGN: A prospective cohort study was performed using data collected from 70 centers registered in the Korean Neonatal Network. Data from the VLBWIs with major congenital anomalies (n = 289) and the controls (n = 867), selected by 1:3 frequency matching for gestational age, were compared. RESULTS: The overall prevalence of major congenital anomalies in VLBWIs was 34.9 per 1,000 live births (289/8,156). The top 2 ranked subgroups of congenital anomalies were the digestive system (31.7%) and congenital heart defects (27.7%), followed by chromosomal anomalies, genitourinary tract defect, central nervous system, other anomalies, undefined, and respiratory system. The group with congenital anomalies had a higher mortality (40.7%) than the control group (11.1%). Each subgroup of congenital anomalies, except for chromosomal anomalies, increased the risk of mortality, with the highest odds ratio associated with "other" anomalies, which includes hydrops fetalis and congenital diaphragmatic hernia. In the multivariate analysis, congenital anomaly was a risk factor for mortality, bronchopulmonary dysplasia, and severe-grade intraventricular hemorrhage. VLBWIs with congenital anomaly demonstrated impaired in-hospital growth as compared with the control group. CONCLUSION: Congenital anomaly increased the risk of in-hospital mortality and was associated with short-term neonatal morbidities in the VLBWIs.

Deep neuromuscular block improves angiographic image quality during endovascular coiling of unruptured cerebral aneurysm: a randomized clinical trial.

BACKGROUND: Neuromuscular block (NMB) used during general anesthesia induces transient skeletal muscle paralysis, but patient movements during endovascular coiling still occur to some degree. Compared with moderate NMB, deep NMB may further improve the intervention condition during endovascular coiling for unruptured cerebral aneurysms; however, little research has focused on the angiographic image quality. METHODS: This prospective, randomized, double-blind clinical trial included 58 patients treated for unruptured cerebral aneurysms with endovascular coiling under general anesthesia. Patients were randomly allocated to either the deep NMB group (post-tetanic count 1 or 2) or the moderate NMB group (train-of-four 1 or 2). The primary outcome was the proportion of patients with a satisfactory intervention condition assessed by surgeons after the procedure using a 5-point intervention condition rating scale (ICRS) from 1 (unable to obtain image) to 5 (optimal); ICRS 5 was defined as satisfactory. RESULTS: There were significantly more cases of satisfactory intervention condition in the deep NMB group than in the moderate NMB group (82.1% vs 51.7%, p=0.015). The frequency of each ICRS score was significantly different between the groups (ICRS 5/4/3/2/1: 23/5/0/0/0 vs 15/9/2/3/0, p=0.035). The incidence of major patient movement requiring rescue muscle relaxant was 10.3% in the moderate NMB group and 0% in the deep NMB group (p=0.237). The drugs used to maintain hemodynamic stability were not significantly different between the two groups. CONCLUSIONS: Deep NMB improves the intervention condition during endovascular coiling by improving the image quality.

A retrospective study on the effects of exclusive donor human milk feeding in a short period after birth on morbidity and growth of preterm infants during hospitalization.

The risks and benefits of feeding preterm formula (PF) versus donor human milk (DHM) in preterm infants are uncertain, and studies evaluating the efficacy of DHM to the morbidities and growth of preterm infants in the neonatal intensive care unit are confused by the need for milk fortification. We aimed to determine and compare the outcome of short-term morbidities in neonatal intensive care unit and growth between premature infants fed exclusively DHM only until a volume of 130 mL/kg/d of enteral feeding was achieved and infants fed with a PF mix after birth. The data of 132 infants with low birth weight of <1500 g and gestational age of less than 32 weeks were considered. Ninety infants were analyzed, of which 86 were discharged alive. The DHM group (n = 36) was made up of infants who were fed exclusively with DHM, whereas the PF group (n = 54) consisted of infants who were fed with a combination of PF and either DHM or human milk, until a volume of 130 mL/kg/d of enteral feeding was achieved. Once feeding in the DHM group progressed to volumes greater than 130 mL/kg/d, infants were fed fortified DHM and PF alternately. One infant (2.8%) in the DHM group had late-onset sepsis or necrotizing enterocolitis compared with 21 (38.9%) in the PF group (adjusted odds ratio 0.05, 95% confidence interval 0.01-0.41); 13 (36.1%) infants in the DHM group had bronchopulmonary dysplasia compared with 38 (70.4%) in the PF group (odds ratio 0.18, 95% confidence interval 0.05-0.41). Although the DHM group demonstrated a comparatively lower rate of weight gain, head circumference increment, and height increment from birth to the age at which an enteral feeding volume of 130 mL/kg/d was achieved, there were no significant differences in these values at 36 weeks' postmenstrual age between both groups.

Treprostinil for persistent pulmonary hypertension of the newborn, with early onset sepsis in preterm infant: 2 Case reports.

RATIONALE: Persistent pulmonary hypertension of the newborn (PPHN) is a syndrome of failed circulatory adaptation at birth with persisting increased pulmonary vascular resistance that is associated with high mortality rates especially in preterm infants. PATIENT CONCERNS: We reported 2 cases of PPHN in preterm infants with respiratory distress syndrome and early onset sepsis refractory to therapy with vasopressors, inotropes, and inhaled nitric oxide (iNO), in whom treatment with treprostinil was successful. DIAGNOSES: Infants showed a difference of more than 10% between pre- and postductal saturation of peripheral oxygen by pulse oximetry. Echocardiogram showed flattened ventricular septum, right to left shunting through the patent ductus arteriosus, and tricuspid regurgitation velocity above 2.9 m/s. INTERVENTIONS: The patients received treprostinil through central venous line because iNO therapy was not effective. OUTCOMES: Within 6 to 12 hours after treatment with treprostinil, the patients showed dramatic clinical improvement, and no systemic side effects were observed, including intraventricular hemorrhage (≥grade II). LESSONS: IV treprostinil might be given to preterm infants with severe PPHN, who did not respond to conservative therapies, including iNO.

A Combination of Short and Simple Surfactant Protein B and C Analogues as a New Synthetic Surfactant: In Vitro and Animal Experiments.

PURPOSE: Pulmonary surfactants for preterm infants contain mostly animal-derived surfactant proteins (SPs), which are essential for lowering surface tension. We prepared artificial pulmonary surfactants using synthetic human SP analogs and performed in vitro and in vivo experiments. MATERIALS AND METHODS: We synthesized peptide analogues that resemble human SP-B (RMLPQLVCRLVLRCSMD) and SP-C (CPVHLKRLLLLLLLLLLLLLLLL). Dipalmitoylphosphatidylcholine (DPPC), phosphatidylglycerol (PG), and palmitic acid (PA) were added and mixed in lyophilized to render powdered surfactant. Synsurf-1 was composed of DPPC:PG:PA:SP-B (75:25:10:3, w/w); Synsurf-2 was composed of DPPC:PG:PA:SP-C (75:25:10:3, w/w); and Synsurf-3 was composed of DPPC:PG:PA:SP-B:SP-C (75:25:10:3:3, w/w). We performed in vitro study to compare the physical characteristics using pulsating bubble surfactometer and modified Wilhelmy balance test. Surface spreading and adsorption test of the surfactant preparations were measured. In vivo test was performed using term and preterm rabbit pups. Pressure-volume curves were generated during the deflation phase. Histologic findings were examined. RESULTS: Pulsating bubble surfactometer readings revealed following minimum and maximum surface tension (mN/m) at 5 minutes: Surfacten® (5.5±0.4, 32.8±1.6), Synsurf-1 (16.7±0.6, 28.7±1.5), Synsurf-2 (7.9±1.0, 33.1±1.6), and Synsurf-3 (7.1±0.8, 34.5±1.0). Surface spreading rates were as follows: Surfacten® (27 mN/m), Synsurf-1 (43 mN/m), Synsurf-2 (27 mN/m), and Synsurf-3 (27 mN/m). Surface adsorption rate results were as follows: Surfacten® (28 mN/m), Synsurf-1 (35 mN/m), Synsurf-2 (29 mN/m), and Synsurf-3 (27 mN/m). The deflation curves were best for Synsurf-3; those for Synsurf-2 were better than those for Surfacten®. Synsurf-1 was the worst surfactant preparation. Microscopic examination showed the largest aerated area of the alveoli in the Synsurf-3 group, followed by Synsurf-1 and Surfacten®; Synsurf-2 was the smallest. CONCLUSION: Synsurf-3 containing both SP-B and SP-C synthetic analogs showed comparable and better efficacy than commercially used Surfacten® in lowering surface tension, pressure-volume curves, and tissue aerated area of the alveoli.

Does an Injection of Adipose-Derived Mesenchymal Stem Cells Loaded in Fibrin Glue Influence Rotator Cuff Repair Outcomes? A Clinical and Magnetic Resonance Imaging Study.

BACKGROUND: The mesenchymal stem cell (MSC)-based tissue engineering approach has been developed to improve the treatment of rotator cuff tears. Hypothesis/Purpose: The purpose was to determine the effect of an injection of adipose-derived MSCs loaded in fibrin glue during arthroscopic rotator cuff repair on clinical outcomes and to evaluate its effect on structural integrity using magnetic resonance imaging (MRI). The hypothesis was that the application of adipose-derived MSCs would improve outcomes after the surgical repair of a rotator cuff tear. STUDY DESIGN: Cohort study; Level of evidence, 3. METHODS: Among 182 patients treated with arthroscopic surgery for a rotator cuff tear, 35 patients treated with arthroscopic rotator cuff repair alone (conventional group) were matched with 35 patients who underwent arthroscopic rotator cuff repair with an injection of adipose-derived MSCs loaded in fibrin glue (injection group) based on sex, age, and lesion size. Outcomes were assessed with respect to the visual analog scale (VAS) for pain, range of motion (ROM) (including forward flexion, external rotation at the side, and internal rotation at the back), and functional measures of the Constant score and University of California, Los Angeles (UCLA) shoulder rating scale. Repaired tendon structural integrity was assessed by using MRI at a minimum of 12 months after surgery, and the mean clinical follow-up was 28.8 ± 4.2 months in the conventional group and 28.3 ± 3.8 months in the injection group. RESULTS: The mean VAS score at rest and during motion improved significantly in both groups after surgery. However, there were no significant differences between the groups at the final follow-up ( P = .256 and .776, respectively). Compared with preoperative measurements, forward flexion and external rotation at the side significantly improved at the final follow-up in both groups (all P < .05). However, no significant improvements in internal rotation at the back were observed in either group ( P = .625 and .834 for the conventional and injection groups, respectively). There were also no significant differences between the groups at the final follow-up for any of the 3 ROM positions (all P > .05). The mean Constant score and UCLA score improved significantly in both groups after surgery, but there were no significant differences between the groups at the final follow-up ( P = .634 and .302, respectively). MRI indicated a retear rate of 28.5% in the conventional group and 14.3% in the injection group ( P < .001). CONCLUSION: This study revealed that an injection of adipose-derived MSCs loaded in fibrin glue during rotator cuff repair could significantly improve structural outcomes in terms of the retear rate. There were, however, no clinical differences in the 28-month period of follow-up. Although still in the early stages of application, MSC augmentation of surgical rotator cuff repair appears useful for providing an adequate biological environment around the repair site.

Decreased Cystatin C-Estimated Glomerular Filtration Rate Is Correlated with Prolonged Hospital Stay in Transient Tachypnea of Newborn Infants.

BACKGROUND: Transient tachypnea of the newborn (TTN) is a benign disorder with a variable clinical course that often leads to hospitalization. The aim of this study was to assess and validate the relationship between the serum cystatin C level and symptom duration in infants with TTN. METHODS: Forty newborns presenting with TTN and who had undergone serum cystatin C (Cys C) tests on the first day of admission to the Kyung Hee University Hospital (Seoul, Korea) from 2009 to 2013 were included. The serum Cys C level, creatinine (Cr) level, estimated glomerular filtration rate (eGFR), and tachypnea duration were correlated retrospectively. RESULTS: The median gestation period was 37.8 ± 3.8 weeks and the mean birth weight was 3.2 ± 0.4 kg. Tachypnea duration was 3.3 ± 2.0 days. Serum Cys C and Cr levels were 1.7 ± 0.2 mg/L and 0.8 ± 1.2 mg/dL, respectively. Tachypnea duration was significantly positively correlated with the serum levels of Cys C and significantly negatively correlated with Cys C-based eGFR (p = 0.016), but was not significantly correlated with the serum Cr level or Cr-based eGFR. When tachypnea duration was compared between infants with Cys C level <1.6 mg/L (n = 15; Group A) and infants with Cys C level ≥ 1.6 mg/L (n = 25; Group B), the symptom duration was significantly shorter in Group A infants (p = 0.011). CONCLUSION: Tachypnea duration was shorter with higher Cys C-based eGFR in infants with TTN.

Development of a Synthetic Surfactant Using a Surfactant Protein-C Peptide Analog: In Vitro Studies of Surface Physical Properties.

PURPOSE: Pulmonary surfactant (PS) replacement has been the gold standard therapy for neonatal respiratory distress syndrome; however, almost all commercial PSs contain animal proteins. We prepared a synthetic PS by using a human surfactant protein (SP) analog and evaluated its in vitro properties. MATERIALS AND METHODS: A peptide sequence (CPVHLKRLLLLLLLLLLLLLLLL) of human SP-C was chosen to develop the peptide analog (SPa-C). The new synthetic SP-C PS (sSP-C PS) was synthesized from SPa-C, dipalmitoyl phosphatidylcholine, phosphatidyl glycerol, and palmitic acid. Physical properties of the sSP-C PS were evaluated by measuring the maximum and minimum surface tensions (STs), surfactant spreading, and adsorption rate. In addition, we recorded an ST-area diagram. The data obtained on sSP-C PS were subsequently compared with those of purified natural bovine surfactant (PNBS), and the commercial product, Surfacten®. RESULTS: The sSP-C PS and Surfacten® were found to have maximum ST values of 32-33 mN/m, whereas that of PNBS was much lower at 19 mN/m. The minimum ST values of all three products were less than 10 mN/m. The values that were measured for the equilibrium ST of rapidly spreading sSP-C PS, Surfacten®, and PNBS were 27, 27, and 24 mN/m, respectively. The surface adsorptions were found to be the same for all three PSs (20 mN/m). ST-area diagrams of sSP-C PS and Surfacten® revealed similar properties. CONCLUSION: In an in vitro experiment, the physical properties exhibited by sSP-C PS were similar to those of Surfacten®. Further study is required to evaluate the in vivo efficacy.

Early Caffeine Use in Very Low Birth Weight Infants and Neonatal Outcomes: A Systematic Review and Meta-Analysis.

The use of caffeine citrate for treatment of apnea in very low birth weight infants showed short-term and long-term benefits. A systematic review and meta-analysis of the literature was undertaken to document the effect providing caffeine early (0-2 days of life) compared to providing caffeine late (≥3 days of life) in very low birth weight infants on several neonatal outcomes, including bronchopulmonary dysplasia (BPD). We searched MEDLINE, the EMBASE database, the Cochrane Library, and KoreaMed for this meta-analysis. The quality of the included studies was assessed using the Newcastle-Ottawa Scale and Jadad's scale. Studies were included if they examined the effect of the early use of caffeine compared with the late use of caffeine. Two reviewers screened the candidate articles and extracted the data from the full-text of all of the included studies. We included a total of 59,136 participants (range 58,997-59,136; variable in one study) from a total of 5 studies. The risk of death (odds ratio [OR], 0.902; 95% confidence interval [CI], 0.828 to 0.983; P=0.019), bronchopulmonary dysplasia (BPD) (OR, 0.507; 95% CI, 0.396 to 0.648; P<0.001), and BPD or death (OR, 0.526; 95% CI, 0.384 to 0.719; P<0.001) were lower in the early caffeine group. Early caffeine use was not associated with a risk of necrotizing enterocolitis (NEC) and NEC requiring surgery. This meta-analysis suggests that early caffeine use has beneficial effects on neonatal outcomes, including mortality and BPD, without increasing the risk of NEC.

Dermatomyositis associated with cancer of unknown primary site.

Dermatomyositis (DM) is an uncommon inflammatory myopathy with characteristic rash accompanying, or more often preceding, muscle weakness. There is a well-recognized association between DM and several cancers, such as ovarian cancer, breast cancer, melanoma, colon cancer, and non-Hodgkin lymphoma. We report the first case of cancer of unknown primary site associated with DM. A 62-yr-old woman presented to us with both shoulder painful swelling and facial edema. She was diagnosed previously as cancer of unknown primary site, histologically confirmed with squamous cell carcinoma in a pelvic mass. For the following days, she complained of erythematous face followed by progressive weakness of the proximal muscles of upper and lower limbs. The laboratory tests showed an increased muscle enzyme and acute phase reactants. The electromyogram showed the typical findings of DM. After the treatment with high dose steroid and methotrexate, the proximal motor weakness improved, and she received palliative radiation therapy.