Idiopathic Acquired Factor VIII deficiency presenting with compartment syndrome: A case report and Literature Review
Haemophilia is a disorder that affects the ability of the blood to form clots. The congenital form of the disease is the most prevalent, is inherited as X-linked recessive and it causes deficiency of clotting Factor VIII or IX. clinically it presents with joint bleeding. Its counterpart, acquired haemophilia is a rare condition that usually presents with cutaneous, soft tissue or internal bleeding. The pathophysiology of the disease is centred on the formation of auto antibodies which inactivate factor VIII. Haematologically this is reflected as a prolonged aPTT with normal PT and failure of mixing studies to correct aPTT to more than 50%. To confirm the diagnosis Bethesda assay has to be performed to detect the presence of factor inhibitors. In half of the cases it is associated with an underlying condition such as autoimmune diseases, malignancy, pregnancy or infections. The mainstay treatment is to control the bleeding with bypassing agents such as recombinant factor VIIa or Factor VIII inhibitor bypassing agent as well as eradicating the inhibitor with immunosuppressive and/or cytotoxic agents. Here we report a patient with idiopathic acquired haemophilia who presented with a thigh compartment syndrome. He was successfully treated with fasciotomy, bypassing agents and immunosuppressive therapy.
Follicular Dendritic Cell Sarcoma of Colon Presenting as Colo-colic Intussusception: a Rare Entity in Gastrointestinal Site With Extremely Rare Presentation
Follicular dendritic cell sarcoma is a rare mesenchymal tumor of follicular dendritic cells arising from nodal and extranodal site. FDCS generally involves the lymph nodes while many extranodal sites are also affected. We report a case of follicular dendritic cell sarcoma primarily arising in colon and presenting as colo-colic intussusception.
Supraglottitis secondary to Haemophilus influenzae serotype f infection: Case report and review of literature
Introduction: Haemophilus influenzae is an important human pathogen capable of causing serious invasive disease. The H. influenzae serotype b (Hib) conjugate vaccine successfully reduced the incidence of invasive Hib disease globally, revealing the significance of other H. influenzae serotypes. The emergence of H. influenzae type f (Hif) invasive disease has been reported worldwide. Case report: We identified a case of supraglottitis secondary to Hif in a 57-year-old female from northwestern Ontario without significant predisposing conditions. We describe the clinical presentation, disease progression, and outcome. The case is discussed in the context of current epidemiology of invasive Hif disease. Conclusion: This report emphasizes the severity of Hif invasive disease and the need for continued surveillance at the international level. We call for the development of health promotion strategies and the consideration of a vaccine against this organism.
Warthin-like variant of papillary thyroid carcinoma (WLV-PTC) is a recently described rare variant of papillary thyroid carcinoma with favorable prognosis. We present a rare case of WLV-PTC in a 29-year-old female patient with graves’ disease along with emphasis on the difficulty of positive diagnosis both clinically and histologically since the association of thyroid nodules with Graves’ disease is not common and this variant of thyroid cancer is frequently misdiagnosed with other subtypes.
Metastasis to the breast from extramammary carcinoma are uncommon. A 63-year-old male presented with abdominal pain resistant to treatment. On clinical examination, the abdomen was soft, depressible but sensitive in the left upper quadrant. Palpation of the right breast revealed a subcutaneous mobile nodule, mobile, measuring 1.5 cm long axis. A whole-body CT was requested and showed a metastatic pancreatic. Mammogram and breast ultrasound showed a relatively well limited nodule of the right breast measuring 22 mm. The patient underwent a breast lumpectomy Final haematoxylin and eosin (HE) and immunochemistry staining of the tumour and immunochemistry confirmed the diagnosis of a metastasis to the breast from pancreatic adenocarcinoma. The patient died within 2 months of initial diagnosis.
We present a patient diagnosed with intrahepatic cholestasis of pregnancy at 30 weeks 6 days of gestation. She presented with complaint of pruritus associated with hepatic cytolysis and elevated transaminases revealed by laboratory tests. The emphasis must be laid on importance of early diagnosis of intrahepatic cholestasis of pregnancy to improve the fetal prognosis and to eliminate other severe conditions associated with hepatic cytoloysis syndrome and pruritus.
The knowledge about the formation and relation of the portal vein is important for surgeons. The variation in the level of formation and the pattern of formation of portal vein is a cause of concern during surgical procedures. An abnormal formation of portal vein that was encountered during routine dissection is reported. The portal vein was formed by the union of splenic vein, superior mesenteric vein and inferior mesenteric veins. Identification of these variations is useful in managing traumatic rupture of the mesentery.
Bronchial Artery Embolization for Life-Threatening Massive Hemoptysis in a young female patient: A case report
Introduction: Massive hemoptysis is a potentially life-threatening respiratory emergency mandating immediate management. It is defined as expectoration of 300 to 600 mL of blood in a period of 24 hours. The most common etiologies include bronchiectasis, cystic fibrosis, neoplasm, sarcoidosis, tuberculosis, and other infections. While only five percent of hemoptysis is massive, some studies report a mortality rate of up to 80 percent in this subgroup, mainly due to asphyxiation. One of the novel ways to manage the later condition is transarterial embolization of the bronchial artery, which is minimally invasive, well tolerated relatively safe procedure. Here we present a case presents with massive hemoptysis due to iatrogenic arteriovenous fistula happened as a complication of previous right middle lobectomy treated successfully by coils. Case presentation: A 25-year-old female patient, a known case of right middle lobectomy 3 years ago due to lobar bronchiectasis presented complaining of less than one day of dyspnea and hemoptysis which was moderate in amount of fresh red blood, there was no fever, cough, recent history of upper respiratory tract infection, chest pain, symptoms of deep vein thrombosis, or anticoagulant use. On examination, the patient was generally ill, conscious, oriented, with average body built, without pallor, not in respiratory distress. She was vitally stable as the blood pressure was 100/65 mmHg, the pulse was 90 beats per minute, respiratory rate was 21 per minute, and temperature was 38.2 C.. Chest examination revealed decreased breath sounds on the right lung, no added sounds and otherwise it was normal. The cardiovascular system examination, neurological examination, abdominal examination, lower limbs examination was normal. Complete blood count was done, and it revealed: hemoglobin level of 14.6 mg/dl, platelet 186.000 per microliter and white blood cells count of 4100 per microliter. Coagulation studies were slightly abnormal as the PT was 15…
Introduction: Large posterior vaginal wall inclusion cysts are rarely reported. According to our review, this case is the largest such cyst reported after posterior vaginal repair. Aim: We present the successful excision of a very large posterior vaginal wall inclusion cyst that developed after posterior vaginal wall repair. Methods: A 44 year old patient presented with a 7 cm symptomatic posterior vaginal wall cyst 5 years after posterior vaginal wall repair. She underwent aspiration of the cyst twice with recurrence before successful surgical excision. Results: The patient recovered without complications. Histopathology revealed a benign inclusion cyst. Conclusions: Although most epidermal inclusion cysts are asymptomatic and can be managed expectantly, cysts that enlarge or become symptomatic should be excised surgically.
Neonate with 10q Interstitial Deletion within the Long Arm of Chromosome 10- A Case Report and Literature Review
Introduction: Partial deletion of distal chromosome 10q was first reported in 1978 by Lewandowski1. Interstitial deletions within bands 10q25e10q26.3 are rare. Seven such cases were reported so far2. Patient Information: A term AGA male newborn was delivered at our perinatal center with antenatal diagnosis of unbalanced translocation of chromosomes 10 and 12, and fetal cleft lip and cleft palate. Blood was sent for chromosome analysis using GTG banding method. Baby had facial dysmorphism, left cleft lip, bilateral cleft of soft and hard palate, intact nasal septum, normal ears and micrognathus. Abdominal ultrasound showed absence of right testis in inguinal canal and abdomen (anorchia). Hospital course was unremarkable except for feeding problems requiring feeding team, plastic surgery planned at 2- 3 months of age, and taping of the cleft lip. He went home on day 7. Conclusion: Here, we reported an extremely rare case of a male newborn with an interstitial deletion within the long arm of chromosome 10 between bands 10q25.1 and 10q26.1, with dysmorphic features, along with a few unreported associations (atypical Pierre-Robin sequence and bilateral dorsal horn ventriculomegaly). We added a comprehensive review of literature on chromosome 10q deletions to the case report. We also listed clinical implications of 71 RefSeq and OMIM genes noted in that ~13.3Mb 10q deletion in the Appendix. Earlier detection of both common and rare chromosomal- genetic abnormalities might prepare the family and health care team to plan optimal care to the mother, and baby.