Maffucci's syndrome is a very rare, congenital and non-hereditary mesodermal dysplasia manifested by multiple enchondromas and soft tissue hemangiomas. Since Maffucci had reported this sysdrome in 1881, there have been more than 100 cases reported, and also there has been reported that Maffuddi's syndrome has various interstitial tumor. However there hasn't been any report about Maffucci's syndrome with testicular teratoma. Here we report in this paper that the patient was diagnosed as Maffucci's syndrome of enchondroma and liver hamangioma and also had testicular teratoma. He was admitted for the evaluation of gynecomatia and diagnosed as enchondroma by bone biopsy of the right rib and tibia. Liver hemangioma was also found through abdominal ultrasonogram, CT scan and liver biopsy. And the testicular teratoma was confirmed through testicular biopsy. He is finally diagnosed as Maffucci's syndrome with testicular teratoma and literatures were reviewed.
Biopsy ; Chondroma ; Enchondromatosis ; Hemangioma ; Humans ; Liver ; Mesoderm ; Ribs ; Teratoma* ; Tibia ; Tomography, X-Ray Computed ; Ultrasonography

In 1947, Ota et al. first reported a case of phakomatosis pigmentovascularis and defined a subgroup of this disease having a congenital generalized hemangioma and pigmented lesions including a mongolian spot-like lesion, nevus pigmentosus and nevus of Ota. We experienced a case of phakomatosis pigmentovasularis type IIb in a 10-day old male baby, who since birth, had generalized nevus flammeus and blue spots, syndactyly between 2nd and 3rd toes in both feet, and dilated collecting system in left kidney.
Foot ; Hemangioma ; Humans ; Kidney ; Male ; Neurocutaneous Syndromes* ; Nevus ; Nevus of Ota ; Parturition ; Port-Wine Stain ; Syndactyly ; Toes

No abstract available.
Herpes Simplex* ; Simplexvirus*

Graft-versus-host disease is commonly observed after allogeneic bone marrow transplantation but rarely recognized after transfusion of solid-organ transplantation. Tansfusion-associated graftversus-host disease can occur in immunosuppressed recipients and immunocompetent transplant recipients. The clinical manifestations of gastrointestinal or hepatic dysfunction, rash and pancytopenia should heighten the physician's index of suspicion for GVHD. Among premature infants, only four cases have been reported to develop transfusion-associated graft-versus-host disease in the world, with a mortality rate of 100 percent. We recently experienced a preterm male infant who developed acute GVHD (erythematous maculopapular skin rash, hepatic dysfunction and pancytopenia) at two months of age and recovered with Dexamethasone and supportive treatment. A skin biopsy, which performed on the confluent erythematous maculopapular rashes during the active and healing stage of the skin rash, revealed characteristic features of GVHD. Prematurity may be considered as a risk factor for the development of GVHD possibly related to complicated prolonged illnesses which requires intensive care and multiple transfusions. To our knowledge, this is the first report of survival after transfusionassociated graft-versus-host disease in preterm infants.
Biopsy ; Bone Marrow Transplantation ; Dexamethasone ; Exanthema ; Graft vs Host Disease* ; Humans ; Infant ; Infant, Newborn ; Infant, Premature* ; Critical Care ; Male ; Mortality ; Pancytopenia ; Risk Factors ; Skin ; Transplantation

PURPOSE: To determine postoperative astigmatic changes after surgery for congenital ptosis and the astigmatic changes depending on surgical method and to discern cases of postoperatively developed anisometropia or amblyopia. METHODS: Fifty-one patients who underwent surgery due to congenital ptosis were reviewed. Refraction or cycloplegic refraction was conducted preoperatively and 3 to 6 months after surgery to measure astigmatic changes. Astigmatic changes in operated eyes were compared to control eyes. Astigmatic changes were compared depending on surgical method. RESULTS: The mean astigmatism was 1.33+/-1.29D preoperatively and 1.48+/-1.13D postoperatively (P=0.10) and the mean astigmatic change in the ptotic and control eye was an increase in cylinder +0.56+/-0.55D and +0.68+/-0.72D (P=0.37). The mean astigmatic change of patients receiving frontalis sling was +0.57+/-0.67D which was similar to those who received levator resection (+0.56+/-0.50D). Newly developed anisometropia was found in three patients postoperatively due to an increase in astigmatism, but newly developed amblyopia was not found. CONCLUSIONS: The increase of astigmatism by ptosis surgery was not statistically significant and there was no statistically significant difference when accounting for surgical method. However, it is necessary to monitor refractive error carefully in younger patients to prevent amblyopia because postoperative increase of astigmatism can cause anisometropia.
Amblyopia ; Anisometropia ; Astigmatism* ; Humans ; Refractive Errors

OBJECTIVE: The STONE score was developed to gauge the ureter stone probability. On the other hand, it is unclear if the STONE score is also applicable to Koreans. This study evaluated whether the STONE score can identify patients with suspected nephrolithiasis for whom a computed tomography (CT) scan can be obtained in the emergency department, targeting Koreans. METHODS: Patients presenting to the emergency medicine (ED) with flank pain and a suspicion of a ureter stone between January 2016 and December 2016 were reviewed retrospectively. After calculating the STONE score, the enrolled patients were classified into the low, moderate, high score group. In each group, the prevalence of a ureter stone and other clinical diseases on a CT scan were investigated. RESULTS: Among the 156 patients included in the analysis, 124 (79.5%) had a ureter stone and an alternative diagnosis was made in 12 (7.7%). The prevalence of a ureter stone and alternative diseases in the high score group was 87.7% (71/81) and 4.9% (4/81), respectively. The proportion of patients with nephrolithiasis increased significantly (P < 0.001), but the proportion of patients with other clinical diseases did not decrease significantly across the score groups (P=0.196). The area under the receiver operating characteristic curve was 0.711 (95% confidence interval, 0.633–0.780; P=0.001) with a cut off score higher than 8 (sensitivity 82.3%, specificity 50.0%) for the STONE score. CONCLUSION: In this setting, the STONE score had a low specificity and could not accurately assess a ureter stone. More studies aimed at Koreans are warranted before a CT scan can be limited using the STONE score.
Diagnosis ; Emergency Medicine ; Emergency Service, Hospital ; Flank Pain ; Hand ; Humans ; Nephrolithiasis ; Prevalence ; Retrospective Studies* ; ROC Curve ; Sensitivity and Specificity ; Tomography, X-Ray Computed ; Ureter* ; Ureteral Calculi

Objective To explore the relationship between apoptosis promoting effector molecules caspase-3,caspase-9 and lubar intcrvertebral disc protrusion.Methods 99 of operation patients with lubar intervertebral disc protrusion in their mater nity ward were recruited.Among them,single segment of lubar intervertehral disc protrusion were 70 (Group A),more than one segments of lubar intervertebral disc protrusion were 29(Group B).In addition,40 unrelated healthy people from physi cal examination center were enrolled as controls.Enzyme linked immunosorbent assay (ELISA) was used to examine serum caspase-3 and caspase-9 levels in lubar intervertebral disc protrusion patients.Results The level of caspase-3 in control group,group A and group B,respectively were 11.24±0.41,14.31±0.67 and 17.43±1.86 pmol/L.The caspase-3 activity in each group was statistically significant difference (F=8.47,P＜0.01).The level of caspase-9 in control group,group A and group B respectively were 18.54±2.19,30.57±3.63 and 43.68±5.15 pmol/L.The caspase-9 activity in each group was statistically significant difference (F=7.85,P=0.001).Compared with control group,the caspasc 3 and caspase-9 ac tivity in group A (q=3.08.3.29,all P＜0.05),group B (q=5.78,4.50,all P=0.014) was statistically significant differ ence.The caspase-3 and caspase-9 activity in group A,group B was statistically significant difference (q=3.21,3.22,all P＜0.05).Conclusion The augment of caspase-3 and caspase-9 promoted apoptosis of lubar intervertebral disc protrusion.It was connected with quantity of protrusive segments.The more segments of protrusion,the higher caspase-3 and caspase-9 levels of examination would be.

Objective:To explore the establishment and assessment of the depression model of adult SD rats induced by early maternal infant separation stress combined with adult chronic unpredictable mild stress(CUMS), and to observe the behavior and synaptic protein expression of SD rats.Methods:Twenty-four male rats were randomly divided into three groups: control group (CON group), chronic unpredictable stress group (CUMS group), maternal infant separation combined with chronic unpredictable stress group (MS + CUMS group). The depression model of rats in cums group was established by CUMS, and the depression model of rats in MS + CUMS group was established by maternal infant separation + CUMS. The depression model was evaluated by body mass and appearance observation, sugar water preference experiment, open field test and forced swimming test.Synapse-associated proteins (PSD-95 and synaptophysin (Syn)) of the hippocampus were assayed using Western blot. Syn protein expression was measured by immunohistochemistry.Results:After modeling, compared with CON group, the rats in CUMS group and MS + CUMS group had significantly lower body mass((126.43±3.88) g, (91.04±3.85) g, (69.89±6.67) g; t=5.03, 8.03, both P<0.01), significantly lower sugar water preference((94.21±0.56)%, (79.30±1.13)%, (72.73±1.82)%; t=8.24, 11.87, both P<0.01) and significantly increased immobility time((3.50±0.84)s, (13.59±2.40)s, (15.70±2.97)s; t=3.16, 3.82, both P<0.01). Among them, the body mass and sugar water preference of MS + CUMS group were lower than those of CUMS group ( t=3.00, 3.63; both P<0.01). (2)The center time of the rats in MS+ CUMS group markedly reduced compared with that of CON group((21.41±4.65) s, (8.96±2.37) s; t=2.66, P<0.05). (3)In the immunohistochemistry experiment, compared with the CON group, the percentage of Syn positive area in hippocampus of rats in CUMS group and MS+ CUMS group decreased significantly ((24.42±0.76)%, (14.00±0.95)%, (10.38±1.38)%; t=6.93, 9.33, P<0.01). (4)The results of Western blot showed that compared with CON group, both the expression of PSD-95 ((1.18±0.02), (0.74±0.06), (0.52±0.05), t=6.29, 9.31; both P<0.05) and Syn ((1.12±0.08), (0.95±0.06), (0.90±0.07); t=3.10, 4.04; both P<0.05) in hippocampus of rats in CUMS group and MS+ CUMS group were significantly decreased.And compared with the CUMS group, the expression of PSD-95 in MS+ CUMS group was lower( t=0.93, P<0.05). Conclusion:The method of MS combined with CUMS can effectively induce depression like behavior and decrease the expression of synaptic plasticity related protein of hippocampus in rats.

Background/Aims: We investigated the clinical practice patterns of post-polypectomy colonoscopic surveillance among Korean endoscopists. Methods: In a web-based survey conducted between September and November 2021, participants were asked about their preferred surveillance intervals and the patient age at which surveillance was discontinued. Adherence to the recent guidelines of the U.S. Multi-Society Task Force on Colorectal Cancer (USMSTF) was also analyzed. Results: In total, 196 endoscopists completed the survey. The most preferred first surveillance intervals were: a 5-year interval after the removal of 1–2 tubular adenomas < 10 mm; a 3-year interval after the removal of 3–10 tubular adenomas < 10 mm, adenomas ≥ 10 mm, tubulovillous or villous adenomas, ≤ 20 hyperplastic polyps < 10 mm, 1–4 sessile serrated lesions (SSLs) < 10 mm, hyperplastic polyps or SSLs ≥ 10 mm, and traditional serrated adenomas; and a 1-year interval after the removal of adenomas with highgrade dysplasia, >10 adenomas, 5–10 SSLs, and SSLs with dysplasia. In piecemeal resections of large polyps ( > 20 mm), surveillance colonoscopy was mostly preferred after 1 year for adenomas and 6 months for SSLs. The mean USMSTF guideline adherence rate was 30.7%. The largest proportion of respondents (40.8%–55.1%) discontinued the surveillance at the patient age of 80–84 years. Conclusions A significant discrepancy was observed between the preferred post-polypectomy surveillance intervals and recent international guidelines. Individualized measures are required to increase adherence to the guidelines.

BACKGROUND/AIMS: Orientia -tsutsugamushi infection is an acute febrile disease due to the accidental transmission through human skin of forest dwelling vector Leptotrombidium larva. The authors observed liver dysfunctions in patients diagnosed with tsutsugamushi disease (Scrub typhus) in the past 3 years and report the data in the hope of bringing attention to this disease in the differential diagnosis of autumn-season hepatitis, especially of non-A, non-B and non-C hepatitis. METHODS: Medical records of 22 patients diagnosed with tsutsugamushi disease by the hemagglutinin method between October 2000 and November 2002 were reviewed. RESULTS: Female gender was dominant in the ratio of 3.4:1. Mean age was 56.4 +/- 2.6. Admission was between 23rd September and 15th November with the peak between mid October and early November. Fever, being the most common symptom, was observed in 21 cases, myalgia in 13, arthralgia in 12, chills in 6, and skin rash in 6. An incubation period of 7-9 days was most common (10 cases), 13-15 days (4), 10-12 days (3), within 3 days (3), and 4-6 days (2). Average ALT, AST and GGTP were increased to 93.2 +/- 17.3 IU/L (18 +/- 345 IU/L), 92.5 +/- 11.7 IU/L (34-255 IU/L) and 132.2 +/- 14.5 IU/L (19-251 IU/L), respectively, but total bilirubin was normal. All the patients improved with doxycycline therapy. CONCLUSIONS: Since it usually shows liver dysfunction, it is important to take Orientia tsutsugamushi into consideration in differential diagnosis of autumn-season, febrile hepatic disease.
Adult ; Aged ; Diagnosis, Differential ; *Disease Outbreaks ; Hepatitis/*diagnosis/microbiology ; Humans ; Korea/epidemiology ; Male ; Middle Aged ; Scrub Typhus/complications/*diagnosis/epidemiology