Fatality in Kikuchi-Fujimoto disease: A rare phenomenon.

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is an uncommon condition, typically characterized by lymphadenopathy and fevers. It usually has a benign course; however, it may progress to fatality in extremely rare occasions. The diagnosis is made via lymph node biopsy and histopathology. Our patient was a young female who presented with shortness of breath, fever, and malaise. Physical examination revealed significant cervical and axillary lymphadenopathy. Chest X-ray displayed multilobar pneumonia. She required intubation and mechanical ventilation for progressive respiratory distress. Histopathology of lymph nodes demonstrated variable involvement of patchy areas of necrosis within the paracortex composed of karyorrhectic debris with abundant histiocytes consistent with KFD. After initial stabilization, the patient's condition quickly deteriorated with acute anemia, thrombocytopenia and elevated prothrombin time, partial prothrombin time, and D-dimer levels. Disseminated intravascular coagulopathy (DIC) ensued resulting in the patient's fatality. DIC in KFD is not well understood, but it is an important cause of mortality in patients with aggressive disease.

Marked Direct Hyperbilirubinemia due to Ceftriaxone in an Adult with Sickle Cell Disease.

Drugs are a significant cause of liver injury. Drug-induced liver injury (DILI) can cause acute hepatitis, cholestasis, or a mixed pattern. Ceftriaxone is a commonly used antibiotic and has been associated with reversible biliary sludge, pseudolithiasis, and cholestasis. A 32-year-old male with sickle cell disease was admitted to the hospital for acute sickle cell crisis. On the second day of hospitalization, he developed cough and rhonchi with chest X-ray revealing right middle lobe infiltrates. Ceftriaxone and azithromycin were initiated. Subsequently, he developed conjugated hyperbilirubinemia and mild transaminitis. His total bilirubin trended upwards from 3.3 mg/dL on admission to 17 mg/dL. It was predominantly conjugated bilirubin, with preadmission bilirubin levels of 3-4 mg/dL. His transaminases were mildly elevated as well compared to previous levels. Extensive workup for bilirubin elevation was unremarkable. Ceftriaxone was switched to levofloxacin and the hyperbilirubinemia improved. On ambulatory follow-up, his bilirubin remained below 4 mg/dL. Ceftriaxone may be associated with marked direct hyperbilirubinemia particularly in sickle cell patients with chronic liver chemistry abnormalities. In the case of elevated bilirubin with concomitant ceftriaxone use, elimination of the offending agent should be considered.

Hamman's Syndrome: A Rare Cause of Chest Pain in a Postpartum Patient.

Hamman's syndrome is a rare condition represented by spontaneous pneumomediastinum and subcutaneous emphysema. Excessive Valsalva maneuver during vaginal delivery and excessive retching, coughing, and straining are frequently reported causes. The incidence of Hamman's syndrome is believed to be 1 in 100,000 deliveries. The pathophysiology of this condition is rupture of alveoli and seepage of air through bronchovascular connective tissue. Diffusion of air to subcutaneous tissues results in subcutaneous emphysema. In most cases, it is a benign condition and resolves spontaneously. In life-threatening cases, a cardiac tamponade can ensue. Chest X-ray is a useful early diagnostic technique. We report a case of a twenty-four-year-old female who was diagnosed with Hamman's syndrome after prolonged, exhaustive labor.

Successful management of acute massive pulmonary embolism using Angiovac suction catheter technique in a hemodynamically unstable patient.

Massive pulmonary embolism with hemodynamic instability is a life-threatening condition requiring immediate treatment. Urgent thrombectomy or thrombolysis is commonly used for the treatment of this condition. However, surgery is associated with high mortality rate and many patients have contraindications to thrombolytic therapy and are at high risk for bleeding. Cather-based intervention has gained increasing popularity particularly in patients with contraindication to thrombolytic therapy or at high risk for surgical thrombectomy. Catheter-based thrombus removal can be achieved by many means such as suction, fragmentation, extraction or rheolytic thrombectomy. We present a case of an elderly lady who suffered from acute massive pulmonary embolism with hemodynamic compromise successfully treated with AngioVac catheter system (AngioDynamics, Albany, NY) with full recovery.

Prompt delivery of thrombolytic therapy: experience with chest pain units and emergency medical services.

OBJECTIVE: To reduce the reperfusion time with thrombolytics. METHODS: The study was done at Sheikh Zayed Hospital, Rahimyar Khan, between January and October 2009, and comprised all consecutive patients with ST segment Myocardial Infarction presenting to the hospital in emergency. In phase one, emergency medical services of Rescue 1122 were utilised to minimize transportation time and thrombolytics were instituted in the Emergency Department or the Coronary Care Unit. In Phase II, a chest pain unit was established in the Emergency Department and all patients were thrombolysed there. A proforma describing Total time, Door-to-needle time, demographics, reperfusion criteria, immediate and delayed complications was filled up for each patient. Comparative groups were analysed using Chi-square and Kruskal-Wallis tests, and p < 0.05 was considered statistically significant. RESULTS: A total of 291 patients were enrolled. Of them 15 (5.15%) were rescued by the emergency medical services, while 276 (94.84%) presented themselves or were referred. Mean age was 51 +/- 11.5 years. There were 245 (84%) males. Thirty (10.30%) patients were thrombolysed at the Chest Pain Unit; 216 (74.22%) at the Coronary Care Unit; and 45 (15.46%) in the Emergency Department. Total time was 3:52, 5:29, and 4:55 hours respectively (p = 0.003). Door-to-needle time was significantly reduced in the chest pain unit (p = 0.0001). Total time was minimum in emergency medical services (p = 0.0001). ST segment resolution > 70% was maximum in the chest pain unit (p = 0.0001). CONCLUSION: There was remarkable reduction in Total time utilising emergency medical services and door-to-needle time by establishing the chest pain unit. It is strongly recommended thatsuch units be developed in all districts and tertiary care hospitals as a cost-effective facility.

Management of brachial artery thrombosis post catheterization.

INTRODUCTION: Brachial artery occlusion is a documented complication after catheterization procedures performed via the brachial artery. These are mainly managed conservatively or with surgical thrombo-embolectomy. We present a case with acute brachial artery occlusion successfully managed with balloon angioplasty and thrombolytic therapy. CASE PRESENTATION: A 68-year-old African-American female with a history of aorto-bi-iliac bypass graft presented with acute right upper extremity pain and discoloration after undergoing peripheral catheterization via right brachial artery. We performed emergency angioplasty with lesion reduction from 100% to less than 20% with TIMI grade flow improving from 0 preintervention to 3 postintervention. Postprocedure, the patient was kept on heparin infusion for 24 hours and discharged on aspirin and clopidogrel, with instructions to go to the emergency room in case of bleeding. CONCLUSION: Acute brachial artery thrombosis is a rare complication post cardiac catheterization. Previously, it was managed with surgical thrombo-embolectomy; however, with advancement of interventional procedures, a more conservative approach is preferred as stated by the 2005 American College of Cardiology/American Heart Association guidelines. We followed these guidelines and performed successful percutaneous balloon angioplasty with tPA administration in our patient. Careful literature search revealed that this is the first published case report dealing with the matter of acute brachial thrombosis post catheterization.

Prevalence of long QT syndrome and other cardiac defects in deaf-mute children.

BACKGROUND: Long QT syndrome is considered a fatal disease because of its association with ventricular arrhythmias and sudden cardiac death. Objectives of study were to determine the prevalence of long QT syndrome and other heart diseases, in deaf-mute children. METHODS: A Cross-sectional descriptive study was conducted at Cholistan special education centre and Cardiology department, Sheikh Zayed hospital Rahim Yar Khan, Pakistan in September 2006. A total of 104 congenitally deaf-mute children were assessed. Height, weight and blood pressure measured, 12-lead electrocardiogram done and QTc calculated using Bazette's formula. Children with prolonged QTc underwent 24-hour ambulatory ECG recording. All were auscultated following complete protocol. A child with murmur was further evaluated with colour Doppler echocardiography. Audiometry was performed on all the children and the result interpreted according to WHO recommendations. Diagnosis of LQTS was based on Revised Schwartz criteria. RESULTS: Out of 104 children, 62 were male with mean age 11.89 yrs. The average systolic and diastolic BP was 97/67 mmHg. Average height was 126 Cm. All children had moderate to severe bilateral sensorineural hearing loss (40-80 dB). One child had associated Patent Ductus Arteriosis. Fifteen had an innocent murmur. Prevalence of congenital heart disease was found to be 0.1/1000. Four children had QT interval more than 440 mSec, (range 0.46-0.47 mSec.). Both genders were equally affected. Three children had high probability of LQTS and one had intermediate probability. Screening of family of these 4 patients showed prolonged QT interval in the sibling of one patient. CONCLUSION: Our study highlights the significant prevalence of Jervell Lange-Nielsen Syndrome in Pakistani deaf-mute children, which may be associated to the high level of consanguinity in this region. Awareness of this syndrome among health care providers is needed as timely diagnosis and subsequent treatment may prevent fatal complications.