Journal of Case Reports in Practice
Volume:3 Issue: 1, Jan 2015

Pulse glucocorticoid therapy is pivotal in severe and life threatening forms of rheumatic and immune-mediated conditions. High dose oral prednisolone has numerous adverse effects and should be balanced with the advantages. Minimal effective dose of most drugs warrants minimal systemic and local side effects. There are limited controlled trials to validate optimal or minimal effective dose, duration and interval of pulse therapies. Considering the relative high biologic half-life of pulsed methylprednisolone, we hypothesize that oral high dose prednisolone could be changed to rather low oral dose during monthly pulse intravenous methylprednisolone.

Congenital bone marrow failure syndromes (CBMFS) are generally diagnosed in infancy and early childhood, but may go unrecognized until adulthood. These syndromes are increasingly being diagnosed into adulthood, possibly due to the availability of genetic testing. We describe a young gentleman diagnosed with acute myelogenous leukemia (AML) who underwent induction chemotherapy with resulting persistent pancytopenia and hypocellular bone marrow without any signs of recovery which is usually seen about 4 weeks after induction. He was incidentally found to have near complete fatty replacement of the pancreas on imaging. The constellation of findings was consistent with a CBMFS. The patient was subsequently diagnosed with Shwachman Diamond syndrome.

This is to report a rare case of enterococcal pneumonia and pleural effusion in a 32-year old woman with severe and active dermatomyositis. She developed fever, respiratory failure, and pleuritic chest pain for 12 days before admission to our hospital. Chest X-ray showed increased overall density of the right hemithorax, indicative of pleural effusion and consolidation with air bronchogram. Cultured pleural drainage revealed growth of Enterococcus faecalis. The patient was treated with imipenem and intravenous immunoglobulin (IVIG) and corticosteroid and the patient’s response to treatment was very good. Chest radiography and chest (computed tomography) CT scan after treatment was quite clear. Enterococci rarely cause pneumonia and plural effusion in general population but it can be concluded that in patients with inflammatory myopathic disease who are treated with immunosuppressive drugs may be a factor of respiratory tract infection.

Acute compartment syndrome (ACS) in the upper extremity may present with many different signs and symptoms such as swelling, severe pain or paresthesia. These patients require an emergent fasciotomy to decompress the compartment. It is commonly caused by major traumas however a few cases of spontaneous ACS is associated with diabetes, oral anti-coagulation therapy, hypothyroidism or nephrotic syndrome. The report’s focus is to identify the sign/symptoms of ACS in patients taking anticoagulant therapy and encourage prompt interventions to prevent ischemia and necrosis. A 33-year-old right hand dominant Hispanic male presented to the emergency department with severe pain and swelling in his right forearm. The patient did not recall any trauma to his arm but stated that he was taking Warfarin for a venous thrombosis diagnosed 3 months ago. A venous duplex was performed which revealed a 3.2 x 2.2 x 2.2cm avascular mass in the anteriomedial forearm suggestive of an intra- compartmental hematoma. Although uncommon, it can lead to spontaneous acute compartment syndrome. Studies have suggested the correlation between warfarin usage and spontaneous ACS. The causative agent should be discontinued or managed and an emergent fasciotomy may be needed to prevent further ischemia, septicemia, limb loss or possible death.

Rectovesical fistula is a rare complication of undiagnosed acute appendicitis. This fistula often presents recurrent symptomatic Urinary Tract Infection (UTI). A case of Rectovesical fistula in a 41-year-old male diabetic patient is described. A perforated appendicitis caused the connection between the base of the bladder and the rectum. There was no appendicovesical fistula connecting with the caecum. In this case a primary resection of the involved rectum was done, the fistula tract excised and simple closure of the bladder and appendectomy was performed.

Dyskeratosis congenita (DKC) is a rare bone marrow failure syndrome which is recognized with a clinical triad of hyperpigmentation, nail dystrophy and oral leukoplakia. Two major conditions are known to be responsible for the high mortality rate of DKC which are aplastic anemia and secondary malignancies. We report a case with the classic triad although without any evidence of the well understood fatal background. Keywords: dyskeratosis congenita, bone marrow failure syndrome, hyperpigmentation, nail dystrophy, oral leukoplakia, secondary malignancies.