Role of C- reactive proteins and liver function tests in assessing the severity of dengue fever.

OBJECTIVE: To determine whether C-reactive protein and liver function tests can serve as severity markers for dengue fever. METHODS: The cross-sectional study was conducted in 2015-16 in Karachi and comprised patients with dengue fever visiting a tertiary care hospital. World Health Organisation classifications 1997 and 2009 were used to categorise patients according to clinical signs and symptoms. Receiver Operating Characteristics curve was used to determine discriminative ability and optimum cut-off value of biochemical markers. Comparisons were done through one-way analysis of variance using SPSS 17. RESULTS: Of the 218 patients, 133(61%) were males and 85(39%) were females. The overall mean age was 35.07±15.96 years. Levels of C-reactive protein and total bilirubin were significantly higher for dengue haemorrhagic fever compared to dengue fever; dengue shock syndrome compared to dengue fever; dengue shock syndrome compared to dengue haemorrhagic fever; and dengue shock syndrome compared to dengue fever / dengue haemorrhagic fever (p<0.05 each). Levels of alanine aminotransferase, aspartate aminotransferase and alkaline phosphatase were significantly higher for dengue shock syndrome compared to dengue fever; dengue shock syndrome compared to dengue haemorrhagic fever; and dengue shock syndrome compared to dengue fever / dengue haemorrhagic fever (p<0.05 each). Levels of C-reactive protein, total bilirubin, alanine aminotransferase and alkaline phosphatise in patients with severe dengue were significantly higher compared to non-severe dengue. CONCLUSIONS: C-reactive protein and liver function tests were found to be effective biochemical markers in assessing dengue fever severity.

Primary Synovial Sarcoma of the Mediastinum in an 18-year old male: A case report.

Primary mediastinal synovial sarcomas are very rare occurrences with only a few cases reported in literature to date. We present a similar case in an 18-year old male which proved challenging to diagnose and treat. Radiological imaging and tru-cut biopsy results gave rise to suspicion of an unusual malignancy. The sarcoma under went en bloc resection and subsequent immunohistochemical staining confirmed the diagnosis of synovial sarcoma. The patient was put on adjuvant chemotherapy after surgery to prevent recurrence. The purpose of this case report is to assist oncologists in the diagnosis and clinical management of this rare tumour.

Lutembacher syndrome with mitral valve calcification in a 31-year old male.

Lutembacher syndrome is characterized by a congenital ostium secundum atrial septal defect and an acquired mitral valve stenosis. We present a similar case in a 31-year old male who came in with orthopnoea, central cyanosis and pedal oedema. Examination revealed cardiac murmurs in tricuspid and apical regions. Chest x-ray showed signs of pulmonary congestion and ventricular enlargement. Electrocardiogaphy (ECG) revealed right axis deviation and right bundle branch block along with atrial fibrillation and Transthoracic Echocardiography (TTE) showed abnormal valves (mitral stenosis with calcification and tricuspid regurgitation) and dilated cardiac chambers. The patient was consequently treated with beta-blockers and diuretics and scheduled for valvular and septal repair via open heart surgery. The purpose of this case report is to assist cardiologists in diagnosing this syndrome accurately on the basis of symptoms and investigations.

Superior mesenteric artery syndrome in an 18-year old female: Case report.

We report an unusual case of an 18-year old woman, who presented to Civil Hospital Karachi in May 2016 with complaints of vomiting, abdominal pain, dysphagia, altered bowel habits, loss of appetite and chronic weight loss. On examination, abdomen was found to be soft and non-tender with discomfort on breathing. CT angiogram revealed reduction of aortomesenteric angle and aortomesenteric distance which were both consistent with superior mesenteric artery syndrome. Reduction in angle was thought to be because of weight loss and adipose tissue depletion so patient was started on enteral and parenteral nutritional supplements. Upon seeing little to no improvement, duodenojejunostomy was performed and patient was kept under observation. Nutritional supplements were continued. The after procedure course was uneventful.

Paediatrics primary myelofibrosis and acute stroke: A rare presentation.

Idiopathic Myelofibrosis is a rare myeloproliferative disorder. In children, it becomes even rarer. Herein, we report a case of idiopathic myelofibrosis of a 6-year old male patient who presented with complaints of pallor, petechiae and bleeding from gums. Bone marrow aspirate showed afragmented haemodiluted smears with erythroid and myeloid precursors scattered throughout. Trephine biopsy showed increased background fibrotic activity along with clusters of histiocytes. A diagnosis of paediatric primary myelofibrosis was made on biopsy. The patient also developed a stroke that resulted in left-sided hemiparesis. An MRI suggested an intraparenchymal haemorrhage along with meningeal extramedullary haematopoiesis.

Infantile metachromatic leukodystrophy in an 18 month old girl.

Metachromatic leukodystrophy is a rarely occurring neurodegenerative metabolic disorder with an incidence of 1-9 individuals out of 1,000,000. We present a similar case in an eighteen month old child which was extremely challenging to diagnose. Clinical symptoms suggested motor regression and developmental delay which gave rise to suspicion of a neurodegenerative disorder. An MRI scan of the brain revealed cortical demyelination with tigroid appearance which confirmed the diagnosis of Metachromatic leukodystrophy. Due to the lack of availability of a treatment option like bone marrow transplant, the patient could only be given physiotherapy to help with the musculoskeletal manifestations of the disorder. The purpose of this case report is to identify clinical presentation and classical MRI findings to diagnose MLD in absence of enzyme assay and gene mutation analysis.

Anomalous right coronary artery arising from left coronary cusp with coexisting valvular heart disease.

We report an unusual case, a 50 year old female with an abnormal right coronary artery originating from the left coronary cusp. The patient, who had a history of hypertension presented with chest pain and shortness of breath to the emergency department. She was diagnosed with ischaemic heart disease (IHD) and had hypertension as one of the coronary risk factor. Echocardiography revealed poor progression of R waves. She was scheduled for echocardiography thereafter which revealed severe aortic stenosis with aortic root dilatation. The patient was discharged due to absence of any complications or other anomalies. This case is unique because of the simultaneously presenting valvular pathology, along with the anomalous origin of the right coronary artery which was detected, as an incidental finding, during coronary angiography.