scholarly journals Sirenomelia (Mermaid Syndrome)

2021 ◽  
Vol 4 (2) ◽  
pp. 185-191
Author(s):  
Defrin Defrin ◽  
◽  
Calvindra Leenesa ◽  
Marcella Marcella

Abstract Objective: To report a rare fetal abnormalities case Method: Case report Case: Reported case of a 37-year-old patient came to the emergency room at RSIA Siti Hawa Padang with complaints of inpartu signs in the last 8 hours before entering the hospital. After the examination, the diagnosis was G3P2A0L3 35-36 weeks of preterm parturient active phase of first stage + twice previous CS + breech presentation. Then, the patient was planned for an emergency CS at 07.50 am. Born babies with genitalia form was not identified, BW: 2030 grams, BH: 30 cm, Scoring Apgar: 3/5, there are congenital abnormalities in the lower extremity of the baby like mermaid form. After observing the baby’s room, the baby was declared dead at 09.50 am. Conclusion: Sirenomelia is a rare congenital defect that has a fatal impact, characterized by varying degrees of fusion in the lower limbs, thoracolumbar spinal anomalies, sacrococcygeal agenesis, genitourinary and anorectal atresia. Because of the poor prognosis, management of sirenomelia will be very difficult with unexpected results. Key words : Fetal abnormalities, Sirenomelia, Mermaid syndrome.

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Victor Aboyans ◽  
Minh Hoang Tran ◽  
Claire Salamagne ◽  
Francoise Archambeaud ◽  
Michael H Criqui ◽  
...  

Background: A high ankle-brachial index (ABI >1.40) is associated with poor prognosis. An underlying peripheral arterial disease (PAD) is frequent in diabetic patients with high ABI, although it cannot be adequately diagnosed by the ankle pressure measurement, due to stiff arteries. We hypothesized that in diabetic patients, the poor cardiovascular disease (CVD) prognosis associated with high ABI would depend on the coexistence of masked PAD. Methods: We reviewed the data of 403 consecutive diabetic patients who had a Doppler assessment of their lower limbs between 1999 and 2000. They were classified as “normal” when Doppler waveform patterns (DWP) were normal and ABI within the 0.91–1.39 range, “PAD only” in case of ABI ≤0.90, “stiff only” if ABI ≥1.40 with normal DWP, and “mixed disease” when ABI ≥1.40 with abnormal DWP. Patients were followed until 04/2008. The primary endpoint was the occurrence of any of the following events: death, stroke or myocardial infarction. Results: The patients (age: 65.6 ± 13.2 yrs, 54.6% females; 90.2% type-2 diabetes) were classified as “normal” (14.4%), “PAD only” (48.4%), “stiff only” (16.4%) and “mixed disease” (20.8%). During a mean follow-up of 6.5 years, the event-free survival curves of “PAD only” and “mixed disease” groups showed poorer prognosis than the “stiff only” and “normal” groups (figure ). In a model adjusted for age, sex, diabetes type and duration, traditional CVD risk factors, renal failure and CVD history, only the presence of PAD was significantly associated with the primary endpoint (OR: 3.36 (1.25 – 4.44), p=0.008). Conclusions: In diabetic patients with high ABI (>1.40), only those with an associated PAD have a poorer prognosis.


2008 ◽  
Vol 20 (6) ◽  
pp. 849-853 ◽  
Author(s):  
Nicole M. Ferguson ◽  
Michel Lévy ◽  
José A. Ramos-Vara ◽  
Debra K. Baird ◽  
Ching Ching Wu

Two yearling bull elk ( Cervus elaphus) from the same farm developed anorexia, weight loss, and lameness. On physical examination, both elk were thin and showed diffuse swelling of all lower limbs. Radiographs of the lower limbs showed periosteal thickening of the distal extremities, consistent with hypertrophic osteopathy. Thoracic radiographs indicated the presence of pulmonary nodules. Cytologic evaluations of tracheal washes on both elk were consistent with inflammation. Acid-fast stains on both samples were negative. Because of the poor prognosis, both elk were euthanized. At necropsy, the carpal, metacarpal, tarsal, and metatarsal bones, as well as the radius, ulna, and tibia had thickening of cortical bone. There were multiple encapsulated nodules throughout the lungs, lymph nodes, and kidney, and smaller nodules in the myocardium. On microscopic examination, these nodules contained myriads of hyphae, and immunohistochemistry for Aspergillus sp. was strongly positive. Aspergillus fumigatus was isolated from affected tissue in 1 elk. Necropsy findings in both elk were consistent with disseminated fungal granulomas and periosteal hyperostosis. This case presents the first description of hypertrophic osteopathy in elk. The source of infection was undetermined, but inhalation of spores from contaminated feed or bedding was suspected.


2021 ◽  
Vol 20 ◽  
pp. 153303382110330
Author(s):  
Lulu Yin ◽  
Yan Liu ◽  
Xi Zhang ◽  
Hongbing Lu ◽  
Yang Liu

Intratumor heterogeneity is partly responsible for the poor prognosis of glioblastoma (GBM) patients. In this study, we aimed to assess the effect of different heterogeneous subregions of GBM on overall survival (OS) stratification. A total of 105 GBM patients were retrospectively enrolled and divided into long-term and short-term OS groups. Four MRI sequences, including contrast-enhanced T1-weighted imaging (T1C), T1, T2, and FLAIR, were collected for each patient. Then, 4 heterogeneous subregions, i.e. the region of entire abnormality (rEA), the regions of contrast-enhanced tumor (rCET), necrosis (rNec) and edema/non-contrast-enhanced tumor (rE/nCET), were manually drawn from the 4 MRI sequences. For each subregion, 50 radiomics features were extracted. The stratification performance of 4 heterogeneous subregions, as well as the performances of 4 MRI sequences, was evaluated both alone and in combination. Our results showed that rEA was superior in stratifying long-and short-term OS. For the 4 MRI sequences used in this study, the FLAIR sequence demonstrated the best performance of survival stratification based on the manual delineation of heterogeneous subregions. Our results suggest that heterogeneous subregions of GBMs contain different prognostic information, which should be considered when investigating survival stratification in patients with GBM.


Author(s):  
Juan Tong ◽  
Lei Zhang ◽  
Huilan Liu ◽  
Xiucai Xu ◽  
Changcheng Zheng ◽  
...  

AbstractThis is a retrospective study comparing the effectiveness of umbilical cord blood transplantation (UCBT) and chemotherapy for patients in the first complete remission period for acute myeloid leukemia with KMT2A-MLLT3 rearrangements. A total of 22 patients were included, all of whom achieved first complete remission (CR1) through 1–2 rounds of induction chemotherapy, excluding patients with an early relapse. Twelve patients were treated with UCBT, and 10 patients were treated with chemotherapy after 2 to 4 courses of consolidation therapy. The 3-year overall survival (OS) of the UCBT group was 71.3% (95% CI, 34.4–89.8%), and that of the chemotherapy group was 10% (95% CI, 5.89–37.3%). The OS of the UCBT group was significantly higher than that of the chemotherapy group (P = 0.003). The disease-free survival (DFS) of the UCBT group was 60.8% (95% CI, 25.0–83.6%), which was significantly higher than the 10% (95% CI, 5.72–35.8%) of the chemotherapy group (P = 0.003). The relapse rate of the UCBT group was 23.6% (95% CI, 0–46.8%), and that of the chemotherapy group was 85.4% (95% CI, 35.8–98.4%), which was significantly higher than that of the UCBT group (P < 0.001). The non-relapse mortality (NRM) rate in the UCBT group was 19.8% (95% CI, 0–41.3%), and that in the chemotherapy group was 0.0%. The NRM rate in the UCBT group was higher than that in the chemotherapy group, but there was no significant difference between the two groups (P = 0.272). Two patients in the UCBT group relapsed, two died of acute and chronic GVHD, and one patient developed chronic GVHD 140 days after UCBT and is still alive, so the GVHD-free/relapse-free survival (GRFS) was 50% (95% CI, 17.2–76.1%). AML patients with KMT2A-MLLT3 rearrangements who receive chemotherapy as their consolidation therapy after CR1 have a very poor prognosis. UCBT can overcome the poor prognosis and significantly improve survival, and the GRFS for these patients is very good. We suggest that UCBT is a better choice than chemotherapy for KMT2A-MLLT3 patients.


2013 ◽  
Vol 177 (1) ◽  
pp. 62-65 ◽  
Author(s):  
Ruicheng Wei ◽  
Lijuan Xu ◽  
Na Zhang ◽  
Kai Zhu ◽  
Juhao Yang ◽  
...  

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Jiang Chen ◽  
Hongyu Li ◽  
Wenda Xu ◽  
Xiaozhong Guo

Abstract Background Pancreatic cancer (PC) is a devastating disease that has a poor prognosis and a total 5-year survival rate of around 5%. The poor prognosis of PC is due in part to a lack of suitable biomarkers that can allow early diagnosis. The lysophospholipase autotaxin (ATX) and its product lysophosphatidic acid (LPA) play an essential role in disease progression in PC patients and are associated with increased morbidity in several types of cancer. In this study, we evaluated both the potential role of serum LPA and ATX as diagnostic markers in PC and their prognostic value for PC either alone or in combination with CA19-9. Methods ATX, LPA and CA19-9 levels were evaluated using ELISA of serum obtained from PC patients (n = 114) healthy volunteers (HVs: n = 120) and patients with benign pancreatic diseases (BPDs: n = 94). Results Serum levels of ATX, LPA and CA19-9 in PC patients were substantially higher than that for BPD patients or HVs (p < 0.001). The sensitivity of LPA in early phase PC was 91.74% and the specificity of ATX was 80%. The levels of ATX, LPA and CA19-9 were all substantially higher for early stage PC patients compared to levels in serum from BPD patients and HVs. The diagnostic efficacy of CA19-9 for PC was significantly enhanced by the addition of ATX and LPA (p = 0.0012). Conclusion Measurement of LPA and ATX levels together with CA19-9 levels can be used for early detection of PC and diagnosis of PC in general.


2021 ◽  
Vol 39 (15_suppl) ◽  
pp. e23500-e23500
Author(s):  
Charles Gusho ◽  
Steven Gitelis ◽  
Alan T. Blank

e23500 Background: Dedifferentiated chondrosarcoma (DCS) is a rare and aggressive malignancy with a poor prognosis. The purpose of this investigation was to assess the clinicopathological features and outcomes of DCS patients treated at a single institution. Methods: This study was a retrospective review over a consecutive twenty-year period. Data including treatment details and outcomes were recorded. Results: A total of 16 cases from 2000 to 2018 were reviewed. The median age was 62 years (IQR, 52-69 years) and the majority of DCS arose in the femur (50%, n = 8) and pelvis (25%, n = 4). Fourteen (88%) cases received limb salvage/wide margin resection (n = 13) or intralesional surgery (n = 1). For all DCS, the median estimated overall survival (OS) was 46 months (95% CI, 1-90 months) with both a five and ten-year survival probability of 32%. On Kaplan-Meier analysis there was no difference between operative versus nonoperative management (p = 0.747), surgery alone versus surgery/chemotherapy (p = 0.265), nor surgery alone versus surgery/chemotherapy/radiation (p = 0.698). Conclusions: Our findings confirm the poor prognosis of DCS patients, though with a five-year estimate of 32%, higher than previous literature. Together with existing literature, our data may enable future strategic recommendation of DCS patients.


2021 ◽  
Vol 76 (4) ◽  
pp. 341-350
Author(s):  
Sergey M. Voevodin ◽  
Tatiana V. Shemanaeva ◽  
Alyona V. Serova

Background.Oligohydramnion in the first half of pregnancy, combined with congenital abnormalities in the fetus has objective difficulties in diagnosis. The morphology features and type of defects associated with oligohydramnion, which manifests in the first half of pregnancy, are not sufficiently studied at the present stage. Aims to evaluate the clinical significance of diagnosing oligohydramnion in the first half of pregnancy in women with congenital fetal malformations. Materials and methods.The analysis of the course of pregnancy and perinatal outcomes in 77 women with low water content in combination with congenital malformations of the fetus and 72 patients with a normal amount of amniotic fluid and no congenital malformations of the fetus was performed. The patients of the main group were divided into two subgroups depending on the severity of oligohydramnion: the 1st subgroup (n = 54) patients with severe oligohydramnion and the 2nd subgroup (n = 23) patients with moderate oligohydramnion. The amount of amniotic fluid was determined by 3D/4D ultrasound (1321 weeks of gestation) and the structure of fetal abnormalities associated with oligohydramnion was analyzed. We evaluated perinatal outcomes in women with congenital malformations of the fetus in combination with oligohydramnion and the effect of its severity on the outcome of pregnancy. Results.In the main group (n = 77), fetal abnormalities were detected in patients: urinary system 39 (50.6%), respiratory system 4 (5.2%), heart 1 (1.3%), chromosomal and genetic abnormalities 14 (18.2%), central nervous system 3 (3.9%), osseous system 3 (3.9%), multiple 13 (16.9%). In the main group (n = 77), pregnancy was terminated for medical indications in 47 (61%) cases, in 6 (7.8%) spontaneous miscarriage occurred, in 5 (6.5%) antenatal fetal death. 19 (24.7%) children were born alive, and surgical treatment in the neonatal period was required in 8 (10.4%) cases. In the 1st subgroup (n = 54) in 53 (98.1%) cases, there was a loss of the fetus, in 1 (1.9%) the newborn died on the 9th day. In the 2nd subgroup (n = 23), fetal death occurred in 5 (21.7%) cases, 18 (78.3%) children were born alive, and 8 (44.4%) newborns were operated on in the neonatal period. In the control group, all pregnancies ended with the birth of healthy children. A decrease in ultrasound imaging of internal organs in the fetus was observed when a pregnant woman was obese (BMI more than 35). Conclusions.Oligohydramnion in the first half of pregnancy in combination with fetal malformation should be considered an extremely unfavorable clinical sign for the prognosis of pregnancy and the health of the fetus and newborn. 3D/4D ultrasound scanning allows you to reliably determine oligohydramnion in the first half of pregnancy, and the degree of its severity to assume the nature of complications.


Cancer ◽  
1990 ◽  
Vol 65 (8) ◽  
pp. 1801-1804 ◽  
Author(s):  
J. Buhr ◽  
K.-H. Berghäuser ◽  
H. Morr ◽  
J. Dobroschke ◽  
H. J. Ebner

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