Background: Primary lymphedema occurs as a result of genetic abnormalities of the lymph system. Currently, complex decongestive therapy is accepted as the standard treatment of the lymphedema. In this case presentation, we described the management of bilateral primary lower extremity lymphedema and the use of complex decongestive therapy.
Case Report: A 62 years old female patient had stage III primary lymphedema on her left lower extremity and stage II primary lymphedema on her right lower extremity. The patient, who had morbid obesity, also had untreatable sleep apnea, urinary incontinence, umbilical hernia and hypertension controlled by drugs. She had stage 4 gonarthrosis according to Kellgren – Lawrence classification in her both knees. The patient received complex decongestive therapy as an outpatient.
After 27 sessions of complex decongestive therapy, edema reduced in both lower extremities. Before the treatment started, the patient couldn’t go up and down stairs, get out and had difficulty mobility in the home. But after the treatment, the patient could go up and down 16 stairs by holding the railing, get out by two walking sticks and had less difficulty mobility in the home. However, due to gonarthrosis in her knees, her pain did not diminish.
Conclusion: Complex decongestive therapy is effective in the management of bilateral primary lower extremity lymphedema, which progressed with multiple health conditions.
Glomus tumors are rare, usually benign, lesions that arise most often in the extremities. They are derived from the glomus body and have a propensity to be found in the fingers and toes, and often in the subungual area [1]. They typically demonstrate pinprick sensitivity, cold hypersensitivity and paroxysmal pain [2]. Only two glomus tumors of the scrotum have been reported and we report a third case of this rare tumor.
Rosai-Dorfman disease (RDD) is an idiopathic, benign proliferation of histiocytes that can be present in multiple organs such as lymph node, skin, soft tissue, orbit, central nerve system and bone; however, it rarely occurs in the breast. In general, RDD is a painless, firm and poorly defined lesion, which can radiologically mimic a breast cancer and is therefore an important differential consideration. The diagnosis of breast RDD is challenging, especially on a small biopsy specimen. We report a unique case of breast RDD with a literature review including common presentation, differential diagnosis, and recommended management. A high index of suspicion for this rare entity is essential to render a correct diagnosis, thereby avoiding unnecessary diagnostic tests and treatment.
Uterine torsion is a rare life-threatening event that happens at any age or any gestational age. By definition, it consists of a rotation of more than 45 degrees around the long axis of the uterus. The reported cases have variable presentations. The uterine torsion can happen without any sequelae either for the fetus or the mother. However, fetal and maternal mortalities were also reported in such a case.
We hereby, report the case of a 29-year-old female patient, with previous four Normal Vaginal Deliveries, pregnant with twins, presenting at 36 weeks gestation with an irreducible uterine torsion at the third trimester of her pregnancy complicated by maternal and fetal deaths.
We concluded that the prognosis is improved as long as the management is done rapidly. More data is needed to know about the genetic predilection and the characteristics of imaging workup for a rapid preoperative diagnosis of this condition.
Bleeding diatheses due to platelet-related disorders can present challenges to treating clinicians especially in the context of peri- and post-partum patients in the obstetric setting. TARS is an inherited disorder characterised by reduced bone marrow platelet production, skeletal deformities affecting radii and other limbs; cardiac, renal, and other heterogeneous anomalies may occur. It is caused by co-inheritance of a microdeletion and a nucleotide polymorphism in the RBM8A gene on chromosome 1.
Bleeding phenotype is more severe than platelet numbers might predict especially in infants but improves with age. There is minimal literature regarding impact in pregnancy and puerperium.
We describe management of three pregnancies in the haematology-obstetrics clinic. As platelet counts normally decrease through pregnancy, close monitoring is required in TAR syndrome. No major bleeding was seen antenatally but two required platelet transfusion during labour. No other treatment definitely improves bleeding, although case reports of steroids claim variable success.
Tranexamic acid may be helpful, and thrombopoietin agonists represent a potential future option.
Ms X is a 34 year old para 1 woman who presented at 26+5 weeks’ gestation with fever, neurological symptoms and history of a viral illness. She was treated empirically for bacterial meningitis and transferred to a tertiary maternity hospital. Cerebrospinal fluid (CSF) polymerase chain reaction (PCR) was positive for enteroviral ribonucleic acid (RNA), confirming viral meningitis. Ms X improved clinically and was discharged after six days. A high index of suspicion is required for diagnosis of meningitis in pregnancy. Thorough history, examination and workup is vital for timely treatment. Prognosis in viral meningitis is excellent with no clear adverse fetal or neonatal outcomes.
Posterior reversible encephalopathy syndrome (PRES) is a neurological syndrome with clinical features of altered sensorium, headaches, visual problems and seizures. It has been associated with uncontrolled hypertension (HTN), thrombotic thrombocytopenic purpura (TTP) and immunosuppressive drugs. Rituximab has also been implicated as a cause of PRES that usually occurs after the first dose. We report a case of PRES that occurred after the second dose of Rituximab. A twenty three years old female known case of resistant TTP treated with multiple courses of steroids and plasmapharesis was admitted with renal failure, severe volume overload ad lower respiratory tract infection. She was treated with hemodialysis, intravenous antibiotics, steroids and plasma exchange (PEX).
A 50-years old female presented with dysarthria, inability to swallow and quadriparesis for three weeks. She had rapid correction of her serum sodium (Na) from 99meq/l to 138meq/l within 24 hours 1 week prior to development of these symptoms. She was diagnosed as a case of Osmotic demyelination syndrome (ODS) formerly known as central pontine myelinolysis (CPM) which was confirmed by MRI. She underwent Plasma Exchange (PE) on the 20th day since her symptoms started and underwent 7 cycles of PE with complete neurological recovery. Pt was discharged with ability to ambulate independently and complete recovery of speech and swallowing. Hence, we report that PE is beneficial in chronic ODS.
Thymoglobulin is a rabbit-derived anti-thymocyte antibody directed at T-cells and commonly used for induction immunosuppression therapy in solid organ transplantation, especially in immunologically high risk kidney transplant recipients. Despite its frequent use and efficacy, the heterologous makeup of thymoglobulin can induce the immune system resulting in serum sickness which typically presents with rash, fever, fatigue, and poly-arthralgia in the weeks following drug exposure. ATGAM is another anti-thymocyte antibody, targeting the same epitopes, but differs from thymoglobulin by the animal in which the preparations are generated (equine vs. rabbit). Herein, we present a case of a patient with a known history of thymoglobulin-induced serum sickness, who presented with evidence of acute cellular and vascular rejection at their 12-month post-operative visit. Given their immunologically high risk status, they were successfully treated with ATGAM with improvement in their rejection and kidney function. To the author’s knowledge, this is the first case report of successful administration of ATGAM in a patient with a documented history of thymoglobulin induced serum sickness, demonstrating a possible treatment option for acute rejection in patients with reactions to thymoglobulin.
Background: AngioJetTM rheolytic thrombectomy has been used in the treatment of deep vein thrombosis (DVT) to prevent post-thrombotic syndrome. Though not widely appreciated, it has the potential to cause intravascular haemolysis.
Report: A 37 year old man with no previous medical history presented to his GP with a three week history of progressive right upper limb swelling. Doppler imaging confirmed right upper limb DVT and CT scan demonstrated thoracic outlet syndrome. The patient underwent AngioJetTM thrombectomy followed by IV heparin infusion. Successful revascularisation of the occluded vein was achieved. Overnight he developed haematuria, which was initially attributed to IV heparin. Urinalysis however revealed no red cells or casts. Apart from an Hb drop from 134 to 117 his blood profile and blood film showed no abnormality. He subsequently developed progressive oliguria with marked oedema and acute kidney injury (AKI). His creatinine peaked at 1070umol/l at 96 hours post procedure and he was started on intermittent dialysis. He remained dialysis dependent for 6 days. Ultrasound imaging excluded urinary obstruction. Autoimmune and vasculitic serology were negative. Intravascular haemolysis and haemoglobinuria was confirmed by raised LDH (1714u/L) and low haptoglobin (<0.1units). Direct Coomb’s test, Cold agglutinin test and paroxysmal nocturnal haemoglobinuria screen were negative. The patient’s renal function normalised over 3 months.
Conclusions: The likely cause of this man’s AKI is heme pigment nephropathy from intra-vascular haemolysis. Increased awareness of this condition may allow early identification and intervention to reduce the risk of renal injury from AngioJetTM associated haemolysis.
A 44-year-old G4P2+1 presented to the emergency department on the 10th day following embryo transfer (with two fresh, day 5, blastocysts transferred in a hospital abroad) with the complaints of difficulty breathing, chest discomfort and cough for one day. These symptoms increased on lying on her side and were not related to exertion. She also mentioned having had abdominal discomfort over the preceding few days. On taking a past history, the patient revealed that all her prior pregnancies were the result of IVF treatment and she suffered OHSS with each. Her first pregnancy was a triplet gestation through IVF and complicated by OHSS; followed by her second pregnancy which was an IVF twin gestation also complicated by OHSS with ascites requiring paracentesis. With her third IVF treatment she conceived, had OHSS and miscarried spontaneously. However, these IVF treatments and pregnancies were all managed abroad and no medical records were available.
The article describes the interaction of anorexic patients, hospitalized in the Regional Pilot Psychiatric Service for the treatment of Anorexia in the Molinette Hospital of Turin, with the reading volunteering group. On the basis of said interaction, the Creative Self is searched for in patients and shows into be present in different ways. It seems anyway enhanced by the presence in the reading group.
Priya Mohan*, Sumathi Bavanandam and Sunil Kumar KS
Published on: 10th July, 2017
Obstructive jaundice in children is not uncommon and has diverse etiologies. We report a rare cause of obstructive jaundice, cholangitis and pancreatic mass in a young boy due to eosinophilic cholangiopathy who responded to oral steroids. Presence of peripheral eosinophilia, elevated serum IgE level, radiological imaging and tissue eosinophilia helped in diagnosis. Eosinophilic cholangiopathy with pancreatitis is a benign treatable cause of obstructive jaundice though it can masquerade as malignancy.
Micronodular thymoma is a rare subtype of thymoma with less than 20 cases published in the English literature. These tumours have been reported with thymoma or thymic cyst. The authors describe a new case of micronodular thymoma in a 68-year-old-patient which is well documented and particular by its cystic degeneration which hasn’t been described yet. Micronodular thymoma is a rare variant of thymoma with a challenging diagnosis. Clinicians must be aware of this entity in order not to confuse it with a thymic cyst.
Anemia due to gastrointestinal blood loss can occur due to many conditions and rarely to bowel structural anomalies. We report a 12 years old girl with anemia due to small bowel duplication cyst, posing diagnostic challenge intra operatively. Surgery offered cure without recurrence of bleeding. Common symptoms can be due to a rare surgical condition in practice.
Ameloblastoma is the second most common odontogenic tumor being back only for the odontoma. An unusual case of recurrent peripheral ameloblastoma in the mandible from the site of previous occurrence, reducing oropharyngeal space due compression by lesion. Panoramic radiography not showed presence of lesion, except one step in left side of mandible angle. Multislice CT scans revealed presence of hypoattenuated image, well-defined, histopathological exam suggesting Ameloblastoma Follicular.
Jorge F Cameselle-Teijeiro*, Javier Valdés-Pons, Lucía Cameselle-Cortizo, Isaura Fernández-Pérez, MaríaJosé Lamas-González, Sabela Iglesias-Faustino, Elena Figueiredo Alonso, María-Emilia Cortizo-Torres, María-Concepción Agras-Suárez, Araceli Iglesias-Salgado, Marta Salgado-Costas, Susana Friande-Pereira and Fernando C Schmitt
A histopathological review preliminary of 429 patients diagnosed with tumours of the uterine corpus (TUC) cancer between 1984- 2010 in the Vigo University Hospital Complex (Spain) were evaluated prospectively for over 5 years. Of these 403 (93.9%) were epithelial tumours: 355 (82.7%) were adenocarcinomas of the endometrioid type, 5 (1.1%) mucinous adenocarcinoma, 10 (2.3%) serous adenocarcinoma, 17 (3.9%) clear cell carcinomas, 11 (2.5%) mixed adenocarcinoma, 4 (0.9%) undifferentiated carcinomas and 1 (0.2%) squamous cell carcinomas. A total 20 (4, 6%) were mesenchymal tumours: 4 (0.9%) endometrial stromal sarcoma, 7 (1.6%) Leiomyosarcoma, 9 (2%) Mixed endometrial stromal and smooth muscle tumour. A total 1 (0.2%) were mixed epithelial and mesenchymal tumours: (0.2%) Adenosarcoma 1. And 5 (1.1%) were Metastases from extragenital primary tumour (3 carcinomas of the breast, 1 stomach and 1 colon). The mean age at diagnosis from total series were 65, 4 years (range 28-101 years). Age was clearly related to histologic type: Endometrial stromal sarcoma 46.0 years, Leiomyosarcomas 57.1 years, Adenocarcinomas of the endometrioid type 65.4 years, Clear cell carcinomas 70.1 years and mixed endometrial stromal and smooth muscle tumours 71.2 years. Five-year disease-free survival rates for the entire group were: Endometrial stromal sarcoma 50%, Leiomyosarcomas 28.6%, Adenocarcinomas of the endometrioid type 83.7%, Clear cell carcinomas 64.7% and mixed endometrial stromal and smooth muscle tumours 44.4%. The 5-year disease-free survival rates of patients with Adenocarcinomas of the endometrioid type tumors were 91.4% for grade 1 tumors, 77.5% for grade 2, and 72.7% for grade 3.
In conclusion, we describe 5-year histological and disease-free survival data from a series of 429 patients with TUC, observing similar percentages to those described in the medical literature. The only difference we find with other published series is a slightly lower percentage of serous carcinomas (ESC) that the Western countries but similar to the 3% of all ESC in Japan. Our investigation is focus at the moment on construct genealogical trees for the possible identification of hereditary syndromes and to carry out germline mutation analysis.
The authors describe the unusual case of subungual onychomycosis, due to fluconazole and itraconazole resistant Candida albicans after using the hybrid and acrylic lacquers and nail tips. The etiology of these atypical changes was supported by isolation of the fungus from the nail lesions, and its consistent identification by means of morphological and molecular diagnosis. In the presented case, topical treatment with ciclopirox 8% nail lacquer allow to fight the pathogenic fungus but did not restore the natural appearance of the nails.
Intraparenchymal cysts without communication to the ventricles or the subarachnoid space are named ependymal or epithelial cysts. The estimated ratio of their incidence compared with arachnoid cysts is 1:10. Neurologic deficit can occur when the cyst exerts mass effect on its surroundings. We report a case of cerebral ependymal cyst in a 75-year-old lady who presented with history of headache, vomiting and left incomplete homonymous hemianopsia. Neuroimaging studies showed a large right occipital cyst. She underwent the neurosurgical procedure of marsupialization. Histologic findings and the immunophenotype was consistent with a diagnosis of ependymal cyst. The patient made an excellent recovery after the procedure.
43-year-old lady presented with incidentally discovered liver lesions while she was being managed for her complaints of menorrhagia. CT and MRI showed hepatomegaly with multiple lesions in both lobes of the liver with vascular element in the background of diffuse fatty infiltration. Patient underwent laparoscopic core biopsy. Histopathology showed extensive steatosis, intracytoplasmic giant mitochondria and absence of portal tracts, features highly suggestive of hepatic adenomatosis. IHC staining showed membranous and cytoplasmic positivity in hepatocytes for B-catenin consistent with multiple hepatic adenomatosis. Hepatic adenomatosis is a new clinical entity in the hepatological practice characterized by the presence of 10 or more nodules in the liver known for its major complication of bleeding. Hepatic adenomatosis is managed by regular imaging and resection of large (> 5cm) superficial and painful adenomas along with liver function tests and tumor markers to rule out malignant transformation. However, the potential cure being the liver transplantation.
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