No abstract available.

We report a case of eosinophilic fasciitis occurring in a 48-year-old man who showed tender, edemstous, indurated, and tight skin on the left forearm and elbow joint. Laboratory findings showed peripheral blood eosinophilia and hypergammaglobulinemia. Other laboratory tests were negative or within normal limits including ANA and anti-DNA. Histopathological findings revealed sclerosis of dermis and thickening of fat and fascia with intense infiltrations of lymphocytes, histiocytes and eosinophils. He was treated successfully with oral prednisolone for 2 weeks.
Dermis ; Elbow Joint ; Eosinophilia ; Eosinophils* ; Fascia ; Fasciitis* ; Forearm ; Histiocytes ; Humans ; Hypergammaglobulinemia ; Lymphocytes ; Middle Aged ; Prednisolone ; Sclerosis ; Skin

We report a case of unilateral nevoid telangiectasia associated with acanthosis nigricans in a 20-year old male patient. Unilateral nevoid telangiectasia may be congenital or acquired. The unilateral and dermatomal distribution suggests that distribution of target vessels is fixed and that they are sensitive to estrogen. Estrogens are mainly produced by the ovaries, but they are also produced by peripheral cirornatization of androgen. Because a major site of this conversion is adipose tissue, estrogen can increase in an obese person. Obesity also reverses the metabolism of estrogen and increases the serum insulin level. Therefore it seems that obesity in our patient induces the development of unilateral nevoid telangiectasia and acanthosis nigricans.
Acanthosis Nigricans* ; Adipose Tissue ; Estrogens ; Female ; Humans ; Insulin ; Male ; Metabolism ; Obesity ; Ovary ; Telangiectasis* ; Young Adult

BACKGROUND: Many physicians hesitate to discuss do-not-resuscitate (DNR) orders with patients or family members in critical situations. In the intensive care unit (ICU), delayed DNR decisions could cause unintentional cardiopulmonary resuscitation, patient distress, and substantial cost. We investigated whether the timing of DNR designation affects patient outcome in the medical ICU. METHODS: We enrolled retrospective patients with written DNR orders in a medical ICU (13 bed) from June 1, 2014 to May 31, 2015. The patients were divided into two groups: early DNR patients for whom DNR orders were implemented within 48 h of ICU admission, and late DNR patients for whom DNR orders were implemented more than 48 h after ICU admission. RESULTS: Herein, 354 patients were admitted to the medical ICU and among them, 80 (22.6%) patients had requested DNR orders. Of these patients, 37 (46.3%) had designated DNR orders within 48 hours of ICU admission and 43 (53.7%) patients had designated DNR orders more than 48 hours after ICU admission. Compared with early DNR patients, late DNR patients tended to withhold or withdraw life-sustaining management (18.9% vs. 37.2%, p = 0.072). DNR consent forms were signed by family members instead of the patients. Septic shock was the most common cause of medical ICU admission in both the early and late DNR patients (54.1% vs. 37.2%, p = 0.131). There was no difference in in-hospital mortality (83.8% vs. 81.4%, p = 0.779). Late DNR patients had longer ICU stays than early DNR patients (7.4 ± 8.1 vs. 19.7 ± 19.2, p < 0.001). CONCLUSIONS: Clinical outcomes are not influenced by the time of DNR designation in the medical ICU. The late DNR group is associated with a longer length of ICU stay and a tendency of withholding or withdrawing life-sustaining treatment. However, further studies are needed to clarify the guideline for end-of-life care in critically ill patients.
Advance Directives ; Cardiopulmonary Resuscitation ; Consent Forms ; Critical Illness* ; Hospital Mortality ; Humans ; Intensive Care Units ; Resuscitation Orders* ; Retrospective Studies ; Shock, Septic

Turner's syndrome results from complete or partial monosomy of the X chromosome and is characterized by hypogonadism or related other congenital anomalies in phenotypic females. In these patients, there are failure to develop normal secondary sex characteristics, amenorrhea, or short stature at puberty and the ovaries are reduced to atrophic fibrous strands devoid of ova and follicles(streak gonads). Individuals with this condition are particularly prone to the development of gonadoblastoma. For this reason, the gonads should be early removed and supplemental estrogen therapy given. We experienced a case of Turner's syndrome, 45, XO/46, XY karyotype in a 20-year-old phenotypic female complained an amenorrhea. On the exploratory laparotomy, the right gonadal mass is sevearly adhered to the adjacent organs and measures 8 x 5 x 5 cm in dimension and 75gm in weight and shows multiple foci of hemorrhage with necrosis. The left streak gonad measures 3.5 x 2 x 1.5 cm in dimension and shows multiple foci of calcification. Microscopically, the right gonadal mass reveals malignant mixed germ cell tumor, composed of endodermal sinus tumor, composed of endodermal sinus tumor with dysgerminoma and gonadoblastoma. The left streak gonad consists of mainly dense fibrous connective tissue and shows some foci of calcification associated with gonadoblastoma. On immunohistochemical and special stainings, the cytoplasm and hyalin droplets of the endodermal sinus tumor component reveal strong positivity to the a-fetoprotein and PAS. After removal of both gonads, the serum level of the a-fetoprotein is markedly down from 1742ng/ml to 2.6 ng/ml.
Female ; Humans

No abstract available.
Female ; Hydatidiform Mole* ; Pregnancy ; Trophoblasts*

The deletion 9p syndrome is a well characterized syndrome with about one hundred cases having been reported. Most patients have dysmorphic facial features, cardiac anomalies, and mental retardation. We report on a female infant with micrognathia, corneal opacity, cleft palace, cardiac anomaly, left polycystic kidney, and deletion 9p. Chromosome analysis and fluorescence in situ hybridization (FISH) showed her to have a derived chromosome 9 inherited from a maternal t(9;16) (p22;p13.2) by adjacent I segregation There are few reports of this particular chromosome rearrangement. We review deletion Sp syndrome.
Chromosomes, Human, Pair 9 ; Corneal Opacity ; Female ; Fluorescence ; Humans ; In Situ Hybridization ; Infant ; Intellectual Disability ; Polycystic Kidney Diseases

Traditionally, liver transplantation for hepatocellular carcinoma with portal vein tumor thrombosis is not recommended. However, with recent developments in locoregional therapies for hepatocellular carcinoma, more aggressive treatments have been attempted for advanced hepatocellular carcinoma. Recently, various studies on locoregional therapies for downstaging followed by living donor liver transplantation reported inspiring overall survival and recurrence-free survival of patients. These downstaging procedures included three-dimensional conformal radiation therapy, trans-arterial chemoembolization, stereotactic body radiation therapy, trans-arterial radioembolization, hepatic arterial infusion chemotherapy and combinations of these therapies. Selection of the optimal downstaging protocol should depend on tumor location, biology and background liver status. The risk factors affecting outcome include pre-downstaging alpha-fetoprotein values, delta alpha-fetoprotein values, disappearance of portal vein tumor thrombosis on imaging and meeting the Milan criteria or not after downstaging. For hepatocellular carcinoma with portal vein tumor thrombosis, downstaging procedure with liver transplantation in mind would be helpful. If the reaction of the downstaged tumor is good, liver transplantation may be performed.

Liver transplantation (LT) is one of the most effective treatments for hepatocellular carcinoma (HCC). Although LT eliminates HCC and greatly reduces recurrence, some patients experience recurrence after LT. Criteria and models for screening patients with a high probability of HCC recurrence after LT, starting with the Milan criteria, have been published. These models have changed over time, but a standard has not been established. We summarized HCC prediction models after LT by focusing on the application of radiologic, serologic, and pathologic factors and recent trends. This review will look at studies that are based on living donor LT and deceased donor LT, as well as studies that downstaging procedures have been performed preoperatively. This ultimately aims to help make decisions for evaluating the HCC state and selecting candidates for LT according to the circumstances of each transplantation center.

Traditionally, liver transplantation for hepatocellular carcinoma with portal vein tumor thrombosis is not recommended. However, with recent developments in locoregional therapies for hepatocellular carcinoma, more aggressive treatments have been attempted for advanced hepatocellular carcinoma. Recently, various studies on locoregional therapies for downstaging followed by living donor liver transplantation reported inspiring overall survival and recurrence-free survival of patients. These downstaging procedures included three-dimensional conformal radiation therapy, trans-arterial chemoembolization, stereotactic body radiation therapy, trans-arterial radioembolization, hepatic arterial infusion chemotherapy and combinations of these therapies. Selection of the optimal downstaging protocol should depend on tumor location, biology and background liver status. The risk factors affecting outcome include pre-downstaging alpha-fetoprotein values, delta alpha-fetoprotein values, disappearance of portal vein tumor thrombosis on imaging and meeting the Milan criteria or not after downstaging. For hepatocellular carcinoma with portal vein tumor thrombosis, downstaging procedure with liver transplantation in mind would be helpful. If the reaction of the downstaged tumor is good, liver transplantation may be performed.