scholarly journals A Case of Newly Diagnosed Klippel Trenaunay Weber Syndrome Presenting with Nephrotic Syndrome

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Egemen Cebeci ◽  
Secil Demir ◽  
Meltem Gursu ◽  
Abdullah Sumnu ◽  
Mehmet Yamak ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Soft Tissues ◽  
Case Reports ◽  
Renal Involvement ◽  
The Body ◽  
Laboratory Findings ◽  
Weber Syndrome ◽  
Av Malformation ◽  
The Right ◽  
Physical Findings

Klippel Trenaunay Weber syndrome (KTWS) is a rare disease characterized by hemihypertrophy, variceal enlargement of the veins, and arteriovenous (AV) malformations. Renal involvement in KTWS is not known except in rare case reports. Herein, we present a case of KTWS with nephrotic syndrome. A 52-year-old male was admitted due to dyspnea and swelling of the body for the last three months. The pathological physical findings were diffuse edema, decreased lung sounds at the right basal site, increased diameter and decreased length of the left leg compared with the right one, diffuse variceal enlargements, and a few hemangiomatous lesions on the left leg. The pathological laboratory findings were hypoalbuminemia, hyperlipidemia, increased creatinine level (1.23 mg/dL), and proteinuria (7.6 g/day). Radiographic pathological findings were cystic lesions in the liver, spleen, and kidneys, splenomegaly, AV malformation on the left posterolateral thigh, and hypertrophy of the soft tissues of the proximal left leg. He was diagnosed to have KTWS with these findings. Renal biopsy was performed to determine the cause of nephrotic syndrome. The pathologic examination was consistent with focal segmental sclerosis (FSGS). He was started on oral methylprednisolone at the dosage of 1 mg/kg and began to be followedup in the nephrology outpatient clinic.

A case of peripheral primitive neuroectodermal tumor of the ovary

2004 ◽  
Vol 14 (2) ◽  
pp. 370-372 ◽  
Author(s):  
K.-J. Kim ◽  
B.-W. Jang ◽  
S.-K. Lee ◽  
B.-K. Kim ◽  
S.-L. Nam
Keyword(s):  
Primitive Neuroectodermal Tumor ◽  
Soft Tissues ◽  
Case Reports ◽  
The Body ◽  
Neuroectodermal Tumor ◽  
Pelvic Cavity ◽  
Age Group ◽  
Peripheral Primitive Neuroectodermal Tumor ◽  
Children And Young Adults ◽  
The Right

Peripheral primitive neuroectodermal tumor (PNET) belongs to the PNET/Ewing's sarcoma family. PNET is a small round cell tumor of putative neuroectoderm origin and is the second most common sarcoma among children and young adults. It may occur anywhere in the body and within any age group; however, it is most likely to occur in the bone and soft tissues. There have been a small number of case reports of PNET arising in the ovary. We presented a case of PNET arising in the right ovary of an 18-year-old woman. The tumor was metastased to the lymph nodes of the pelvis and para-aorta at surgical staging. We had persecuted Taxol/carboplatin chemotherapy, pelvic cavity radiotherapy, and Vincristine/Actinomycink, Cyclophosphamide/Doxorubicin (VACA). She died after 10 months due to septic shock.

scholarly journals Report of a newborn with hemimegalencephaly in association with Klippel-Trenaunay-Weber syndrome

2005 ◽  
Vol 133 (1-2) ◽  
pp. 62-64
Author(s):  
Slobodan Obradovic ◽  
Biljana Vuletic ◽  
Andjelka Stojkovic-Andjelkovic ◽  
Zoran Igrutinovic
Keyword(s):  
Case Report ◽  
Soft Tissues ◽  
Varicose Veins ◽  
The Body ◽  
Arteriovenous Fistulae ◽  
Left Part ◽  
Weber Syndrome

Klippel-Trenaunay-Weber syndrome (KTW) is a rare phacomatosis whose main characteristics are: cutaneous angiomas (vascular nevi), varicose veins (arteriovenous fistulae) and hemihypertrophy of bones and soft tissues. This is a case report of KTW syndrome which was diagnosed in the first days of life on the basis of characteristic vascular nevi on the skin of the trunk and extremities in addition to hypertrophy of the left part of the body. Neuroradiologically verified hemimegalencephaly associated with temporal hemangioma represents a special particularity in our case. Measures of monitoring the child as well as stimulating and therapeutic actions were the issues of discussion.

scholarly journals Atrial thrombus as a complication of SLE and APS in an 8-year-old child

2020 ◽  
Vol 18 (1) ◽  
Author(s):  
Hai-bo Yan ◽  
Yu-mei Li
Keyword(s):  
Nephrotic Syndrome ◽  
Anticoagulant Therapy ◽  
Right Atrium ◽  
Rare Complication ◽  
Renal Involvement ◽  
Clinical Manifestations ◽  
Disease Diagnosis ◽  
Urinary Protein ◽  
Multiple Systems ◽  
The Right

Abstract Background Systemic lupus erythematosus (SLE) is an autoimmune disease involving multiple systems with various clinical manifestations. Renal involvement is common, but intracardiac thrombus is rarely reported as a complication of antiphospholipid syndrome (APS, also known as anticardiolipin syndrome). Anticoagulant therapy is the first-line treatment, and surgery is performed in severe cases. We report a case to improve clinicians’ understanding of disease diagnosis. Case presentation An 8-year-old girl was admitted to our hospital because of left costal pain, hematuria and fever. She had obvious edema occult blood 3+, urinary protein 3.2 g/24 h, albumin 17.6 g/L, and total cholesterol 7.21 mmol/L, consistent with a diagnosis of nephrotic syndrome. We continued to track the etiology of nephrotic syndrome and performed a renal biopsy, showing dsDNA 1:10 positivity, low C3, low platelets and hemoglobin, anticardiolipin IgM 12 U/ml, anti-β2-glycoprotein I (β2GPI) 223 U/ml; renal pathology suggested lupus nephritis (LN), and the patient was ultimately diagnosed with SLE, secondary APS and LN. The patient was treated with hormones and immunosuppressants. Sixteen weeks later, her urinary protein was 1+, and the quantity of urine protein was less than 0.5 g/d. Echocardiography showed that the mass in the right atrium was thrombotic. Heparin anticoagulant therapy was effective. Conclusion SLE can involve multiple systems and various complications. Thrombus in the right atrium is a rare complication of APS. Early diagnosis and treatment are key to improving the prognosis of children.

Calcifying fibrous tumour originating from the right cardiac ventricle in a child

2013 ◽  
Vol 24 (1) ◽  
pp. 161-163 ◽  
Author(s):  
Ben Zhang ◽  
Weida Zhang ◽  
Xiaowu Wang
Keyword(s):  
Right Ventricle ◽  
Soft Tissues ◽  
The Body ◽  
Dystrophic Calcification ◽  
Cardiac Ventricle ◽  
The Right

AbstractCalcifying fibrous tumour is a rare benign fibrous lesion. It is paucicellular, with fibroblasts, dense collagenisation, psammomatous and dystrophic calcification, and patchy lymphoplasmacytic infiltrates. Calcifying fibrous tumour was first described in subcutaneous and deep soft tissues, and has been reported all over the body. However, calcifying fibrous tumour originating from the heart is extremely rare. This article describes the case of a giant calcifying fibrous tumour arising from the right ventricle in a child, where the tumour was totally resected and no recurrence was observed during a 4-year follow-up period.

scholarly journals A Case Report: A 37 Years-old Women with Takayasu Arteritis

2021 ◽  
pp. 56-60
Author(s):  
Shakib H. Sheikh ◽  
Arti Raut ◽  
Savita Pohekar ◽  
Dipali Ghungrud ◽  
Swapna Morey
Keyword(s):  
Takayasu Arteritis ◽  
The Body ◽  
Her Family ◽  
Lower Segment Caesarean Section ◽  
Disease Condition ◽  
History Of ◽  
The Difference ◽  
Wbc Count ◽  
The Right ◽  
Physical Findings

Background: A rarity of form of vasculitis, also known as TAK, induces inflammation in the walls of the major arteries in the body: the aorta and its main branches. The disease results from a body attack and inflammation of the walls of the arteries caused by the body's own immune system. Case Presentation: A 37-years-old women had complaints of fever, giddiness, weakness of right upper limb and lower limb since1 day. After undergoing whole blood count, liver function examination, renal and MRI function checks, CT scan, angiography, etc. was studied. She was diagnosed with takayasu arteritis.  She had past medical history of neck pain and numbness since January 2020. For these complaints her family members referred her in private hospital. There is no significant history of surgery in present, lower segment caesarean section and piles operation was done previously. Physical findings were normal except the Glasgow Coma Scale score was 11 that is, patient  was semi-conscious, In General appearance patient activity was dull and weak due disease condition, Patient’s all routine investigations were normal except few like Total WBC count was increased (21,500cell /cm) due to increased infection, Granulocytes were increased  that is 75 % due to infection and autoimmune disease, ESR was increased  that is52mm/h. Angiography showed block in the right common carotid artery, MRI- showed Acute infract in left fronto  temporo parietal region involving insular par ventricular white matter,  absent flow in distal M1 segment of MCA. Conclusion: The most frequently involved arteries and the angiographic trends in this Takayasu Arteritis study were subclavian arteries and carotid arteries. The difference between angiographic characteristics may lead to clinical differences. Operations and surgery should be carried out at various points in the course of the disease.

scholarly journals Triple whammy: a rare case of epithelioid hemangioendothelioma with synchronous angiomyomatous hamartoma complicated by Actinomyces meyeri

2021 ◽  
Vol 8 (3) ◽  
pp. 56-61
Author(s):  
Nariman Khan ◽  
◽  
Saadia Waheed ◽  
Rahaf Alkhateb ◽  
◽  
...  
Keyword(s):  
Groin Pain ◽  
Soft Tissues ◽  
Treatment Guidelines ◽  
Epithelioid Hemangioendothelioma ◽  
The Body ◽  
Vascular Endothelial ◽  
Imaging Studies ◽  
One Year ◽  
The Right ◽  
Hispanic Male

Epithelioid hemangioendothelioma is a rare vascular malignancy that originates from vascular endothelial or pre-endothelial cells and is composed of epithelioid or histiocytoid cells. This malignancy has an incidence of approximately one per one million individuals and can occur in various regions of the body including the lungs, liver, bones, and soft tissues. The behavior of this cancer can range from indolent to aggressive and diagnosis and treatment are often delayed due to variable presentations and lack of established treatment guidelines. Here we present the case of a 27-year-old Hispanic male that presented with right groin pain, abdominal pain, and a fifty-pound weight loss over one year. The patient had a complex hospital course during which he was found to have an angiomyomatous hamartoma of his right groin area, postsurgical right inguinal wound infection with Actinomyces meyeri, and epithelioid hemangioendothelioma distal to the right iliac bifurcation. The patient is currently pending further imaging studies to evaluate candidacy for surgical resection and following with oncology for chemotherapeutic options.

scholarly journals Splanchnic venous thrombosis in a nephrotic patient following COVID-19 infection: a case report

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Maged H. Hussein ◽  
Mohamad S. Alabdaljabar ◽  
Noorah Alfagyh ◽  
Mohammad Badran ◽  
Khalid Alamiri
Keyword(s):  
Nephrotic Syndrome ◽  
Case Reports ◽  
Hypercoagulable State ◽  
Hepatic Veins ◽  
Vein Thrombosis ◽  
Abdominal Symptoms ◽  
Small Series ◽  
Steroid Dependent ◽  
Nephrotic Patient ◽  
The Right

Abstract Background As the COVID-19 pandemic spread worldwide, case reports and small series identified its association with an increasing number of medical conditions including a propensity for thrombotic complications. And since the nephrotic syndrome is also a thrombophilic state, its co-occurrence with the SARS-CoV-2 infection is likely to be associated with an even higher risk of thrombosis, particularly in the presence of known or unknown additional risk factors. Lower extremity deep vein thrombosis (DVT) and pulmonary embolism (PE) are the most common manifestations of COVID-19-associated hypercoagulable state with other venous or arterial sites being much less frequently involved. Although splanchnic vein thrombosis (SVT) has been reported to be 25 times less common than usual site venous thromboembolism (VTE) and rarely occurs in nephrotic patients, it can have catastrophic consequences. A small number of SVT cases have been reported in COVID-19 infected patients in spite of their number exceeding 180 million worldwide. Case presentation An unvaccinated young adult male with steroid-dependent nephrotic syndrome (SDNS) who was in a complete nephrotic remission relapsed following contracting SARS-CoV-2 infection and developed abdominal pain and diarrhea. Abdominal US revealed portal vein thrombosis. The patient was anticoagulated, yet the SVT rapidly propagated to involve the spleno-mesenteric, intrahepatic and the right hepatic veins. In spite of mechanical thrombectomy, thrombolytics and anticoagulation, he developed mesenteric ischemia which progressed to gangrene leading to bowel resection and a complicated hospital course. Conclusion Our case highlights the potential for a catastrophic outcome when COVID-19 infection occurs in those with a concomitant hypercoagulable state and reminds us of the need for a careful assessment of abdominal symptoms in SARS-CoV-2 infected patients.

scholarly journals Long-lasting severe knee pain in a SLE patient after renal transplantation: what is the reason? A case report and literature review

2021 ◽  
Author(s):  
Anna Masiak ◽  
Iga Kościńska ◽  
Beata Rutkowska ◽  
Zbigniew Zdrojewski
Keyword(s):  
Kidney Transplantation ◽  
Case Report ◽  
Knee Pain ◽  
Case Reports ◽  
Immunosuppressive Treatment ◽  
The Body ◽  
Review Of The Literature ◽  
Letters To The Editor ◽  
Appropriate Treatment ◽  
The Right

AbstractMusculo-skeletal complaints in a patient suffering from systemic lupus, with co-existing chronic renal failure, undergoing immunosuppressive treatment after kidney transplantation, can have a varied etiology. The aim of this work was to present a case based review of differential diagnosis of knee pain in such a patient. A literature search was carried out using MEDLINE/PubMed, Google Scholar and EBSCO, with no time limit. We undertook a systematic review of the literature published in English, limited to full-text publications of original articles, letters to the editor, and case reports in peer-reviewed journals, for a discussion and analysis of studies reporting arthralgia in patients with lupus after kidney transplantation. We present a case report of a 45-year-old woman with lupus nephritis, after kidney transplantation, who started to complain of increasing pain in the knees, most pronounced at night and after physical activity approximately 2 years after transplantation. Extensive causal diagnostics were carried out, which revealed bilateral extensive regions of bone infarction in the femur and tibia, chondropathy, degenerative changes of medial meniscuses in the body and posterior horn as well as chondromalacia of the patella. Establishing the right diagnosis is crucial for implementing appropriate treatment.

scholarly journals Case Report of a Rare Cause of Reactive Arthritis: Leptospirosis

2021 ◽  
Vol 11 (3) ◽  
Author(s):  
Sudipan Dey ◽  
Arun Kumar Sipani ◽  
Rajdeep Das
Keyword(s):  
Case Report ◽  
Hip Joint ◽  
Reactive Arthritis ◽  
Past History ◽  
Case Reports ◽  
Female Child ◽  
The Body ◽  
Pathogenic Leptospira ◽  
Magnetic Resonance Imaging Mri ◽  
The Right

Introduction: Leptospirosis is a zoonosis caused by infection with pathogenic Leptospira species. Leptospirosis has protean manifestations and rare, unusual presentations should be kept in mind in relevant epidemiological scenario. Reactive arthritis refers to acute non-purulent arthritis complicating an infection elsewhere in the body. It is attributed to an immune activation following certain infections; it is, therefore considered as aseptic arthritis. Very few case reports are available attributing leptospirosis as an established cause of reactive arthritis. We present a case of reactive arthritis of the hip joint due to leptospirosis. Case Report: Here, we present a case of a 12-years- old female child who was admitted to our hospital with complaints of fever, headache, and pain in the right hip joint since past 5 days from admission. Subsequent elaboration revealed a past history of fever, headache, and myalgia for around 5-7 days around a week before the present complaints. There was rat infestation near her house and her father was working as sewage cleaner. Routine investigations, Ultrasonography (USG), Magnetic Resonance Imaging (MRI) of both hips and subsequently, diagnostic hip aspiration was performed. USG revealed synovitis, MRI revealed hip joint arthritis of infective or inflammatory origin. Diagnostic hip aspiration was negative for any microorganism. On 10th day of admission, patient started developing icterus with yellowish discolouration of urine. Patient was evaluated for the cause of jaundice. Screening for Leptospira was positive. Synovial biopsy of hip was performed, which showed inflammation with no specific pathology and no growth of any microorganism. In addition, Leptospira IgM MAC ELISA was done which was positive. Patient was thus confirmed to be having leptospirosis and reactive arthritis as a consequence of it. Conclusion: The presentation of reactive arthritis secondary to leptospirosis is rare. Leptospirosis can be an etiological fa

scholarly journals Primary Intraosseous Synovial Sarcoma with Molecular Confirmation: Expanding and Clarifying the Spectrum of This Rare Neoplasm

2020 ◽  
Vol 2020 ◽  
pp. 1-11
Author(s):  
Kelsey E. McHugh ◽  
John D. Reith ◽  
Nathan W. Mesko ◽  
Scott E. Kilpatrick
Keyword(s):  
Soft Tissue ◽  
Synovial Sarcoma ◽  
Soft Tissues ◽  
Case Reports ◽  
Bone Lesion ◽  
Metastatic Potential ◽  
Metatarsal Bones ◽  
Synovial Sarcomas ◽  
Primary Bone ◽  
The Right

Synovial sarcoma is a well-known malignant tumor usually originating within deep soft tissues of the lower extremities of adolescents and young adults. Rare radiologically confirmed examples of primary bone synovial sarcoma have been documented, generally in isolated case reports. Herein, we report two cases of primary intraosseous synovial sarcoma, with molecular confirmation, involving the left humerus of a 45-year-old female and the right fourth metatarsal bone in a 36-year-old male. Additionally, we clarify the spectrum of primary intraosseous synovial sarcoma by separately analyzing reported cases with radiographic confirmation of bone origin and molecular support for the diagnosis. There are clinicopathologic differences between those tumors with documented molecular confirmation and those lacking such confirmation, specifically regarding their anatomic distribution (p<0.0001). Regarding the radiology of our two cases, the humeral lesion appeared almost entirely intramedullary without soft tissue extension; the midfoot lesion demonstrated a destructive, metatarsal-centered bone lesion, initially thought clinically to represent primary bone osteosarcoma. The diagnoses of monophasic synovial sarcoma were rendered via core needle biopsies, with molecular FISH confirmation of SYT gene rearrangement. Clinical follow-up data was only available for the female patient with the primary humeral lesion, who underwent surgical resection, with no local recurrence or distant metastasis at 7 months postsurgery. To our knowledge, these are the first reported examples of molecularly confirmed, primary intraosseous synovial sarcomas of the humerus and metatarsal bones. Primary intraosseous synovial sarcomas with molecular confirmation differ clinically from those lacking it; however, the demographic features and metastatic potential appear similar to primary soft tissue synovial sarcoma.

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