Cardiac amyloidosis: state-of-the-art review / 老年心脏病学杂志（英文版）

Cardiac amyloidosis (CA) is caused by deposition of amyloid fibrils in the myocardium and has two main subtypes, transthyretin cardiac amyloidosis (ATTR) and immunoglobulin light chain cardiac amyloidosis (AL). ATTR is further differentiated into wild-type (wtATTR) and hereditary (hATTR), depending on the absence or presence of mutation in the transthyretin gene. The increased recognition of disease with the improvement in diagnostic armamentarium and serendipitous advancements in the therapeutic landscape have changed the status of CA from being a rare and untreatable disease to being a not-so-rare and treatable disease. Both ATTR and AL have certain clinical aspects that can provide early clues for the disease. While electrocardiography followed by echocardiography and subsequently cardiac magnetic resonance can raise suspicion for CA, the definitive diagnosis of ATTR is non-invasively established by bone scintigraphy while that of AL always needs histological confirmation. Severity of CA can be gauged by serum biomarker-based staging of both ATTR and AL. ATTR therapies work by silencing or stabilizing TTR or by degrading amyloid fibrils, while AL is managed with anti-plasma cell therapies and autologous stem cell transplant.

Radiological and clinical outcomes in patients undergoing anterior cervical discectomy and fusion: comparing titanium and peek [polyetheretherketone] cages

Objectives: To study clinical and radiological outcomes in patients who had undergone the procedure of anterior cervical discectomy and fusion with titanium or PEEK [polyetheretherketone] cages for cervical disc prolapse

Methods: This is a retrospective/non-randomized study which was conducted at the Combined Military Hospital Peshawar. Study interval was four years from 1st October, 2010 to 31st September, 2014. Total number of included patients were 149. All of the patients had undergone the procedure of anterior cervical discectomy and fusion with titanium or PEEK [polyetheretherketone] cages. All of the patients had plain MRI cervical spine done for diagnosis of anterior cervical disc prolapse

Results: Most of the patients had stenosis at the C5 / C6 [PEEK cage group 63% and titanium cage group 47.6%] and C6 / C7 [PEEK cage group 15.38% and titanium cage group 19.04%] cervical level. Bi-level involvement was also seen. In the patients who complained of brachialgia, total resolution of symptoms was seen after the operation. Three [2.01%] of the patients in titanium cage group, who presented with axial neck pain, continued to complain of pain after the operation. Four [2.6%] of the patients in PEEK [polyetheretherketone] cage group and 2 [1.3%] in titanium cage group complained of pain at the donor site [iliac crest]. Fusion rate was 100% with both titanium and PEEK [polyetheretherketone] cages at one year

Conclusion: Results with titanium and PEEK [polyetheretherketone] cages are excellent. There was no significant difference in clinical and radiological outcome between two groups of patients [p > 0.05]. Fusion rate was 100% at one year with both cages

Comparison between extra oral and intraoral surgical procedures for the management of mandibular angle fractures

Objective: To evaluate and compare the treatment outcomes of isolated mandibular angle fractures managed by open reduction and internal fixation using intra oral and extra oral approaches. Study Design: Randomized control trial. Place and duration of Study: Department of oral and maxillofacial surgery, Liaquat University Hospital Hyderabad, from May 2011 to Nov 2011

Patients and Methods: The patients who presented with isolated mandibular fracture were selected according to inclusion criteria. The diagnosis was made by standard history, clinical examination and radiographic investigations. The patients were randomly divided into two groups, A and B. Group A patients were managed by extra oral approach and group B patients were managed using intra oral approach. The patients were prescribed antibiotics for one week and follow up was carried for four weeks. Nerve damage, limited mouth opening, infection, mal-occlusion, hypertrophic scar and aesthetic dissatisfaction were immediate and late post operative complications observed and documented

Results: Road traffic accident [RTA] was the main etiologic factor [66.66%]. Marginal mandibular nerve damage was noted in 20% cases treated with extra oral approach [group A]. Post operative esthetic dissatisfaction was present in 60% patients [group A] as compared to 6.66% of the patients [group B]. The rate of mouth opening compromise and mal-occlusion were also higher in the patients treated with extra oral approach compared with intra oral approach, although statistically not significant

Conclusion: The intra oral approach for mandibular angle fracture management is an effective and comparatively better technique as compare to the extra oral approach

Blood ammonia level predicts the presences of esophageal varices in patients of liver cirrhosis due to hepatitis B and C viruses

Objective: to determine the biochemical and ultra-sonographic noninvasive parameters in liver cirrhotic patients due to Hepatitis Band C virus. To validate the non-invasive parameters with the presence of esophageal varices using endoscopy as gold standard

Material and Methods: two hundred diagnosed patients were taken. Every patient underwent esophagogastroduodenoscopy [EGO] for the presence of esophageal varices. Ultrasonography was done to calculate the spleen diameter. Blood samples were taken to find the levels of blood ammonia and platelet count

Results: on the basis of Receiver operating curve [ROC] blood ammonia level had area under the curve [AUC] 1.000 [p<0.001] platelet count/spleen diameter ratio [AUC=0.008, p<0.001] platelet count [AUC 0.009, p<0.001] and spleen diameter had area under the curve 0.986 [p<0.001] The maximum area under the curve was observed with blood ammonia [100%] as compared to other parameters

Conclusion: it was concluded from the present study that blood ammonia level is the most reliable non-invasive parameter in predicting the presence of esophageal varices as compared to other non-invasive parameters

Clinical presentation of lipoid pneumonia among children

Background: The traditional medicinal use of different fat based remedies for the relief of various common childhood ailments continues to be a public health problem

Objective: To determine the clinical profile of the patients of lipoid pneumonia in Sheikh Zayed Hospital, Rahim Yar Khan

Patients and Methods: This descriptive study was conducted at the department of Pediatrics, Sheikh Zayed Hospital/ Medical College, Rahim Yar Khan. The study period was 02 years, from 1st January 2010 to 31st December 2011. All the patients presented to the department during the study period and diagnosed as lipoid pneumonia were included in the study. In all suspected patients of lipoid pneumonia a detailed history focusing mainly on birth history, feeding history including intake of any oil, ghee, butter and complaints of respiratory system was focused. Thorough physical examination particularly of respiratory system and CNS was carried out. Investigations carried out were CBC, Blood C/S, x-ray chest and CT scan chest

Results: During the study period of 02 years, 22 [0.0006%] cases of lipoid pneumonia were diagnosed on clinical and radiological grounds. In all cases birth history was unremarkable and all patients were developmentally normal. Acute presentation with high grade fever, cough and respiratory distress was noted in 05 [22.7%] cases while the onset was insidious with persistent or recurrent respiratory symptoms in 17 [77.2%] cases. The time interval between aspiration and presentation was <24 hours in 01 [4.5%] case, 2-7 days in 04[18.2%] cases and >7 days to months in 17 [77.2%] cases. Daisee Ghee was administered in 16 [73%] cases, Castor Oil in 04 [18%] cases while Gingilli oil and olive oil in 01 [4.5% each] case each

Conclusion: Lipoid pneumonia be considered in the differential diagnosis of all cases of respiratory distress more particularly of chronic/recurrent respiratory symptoms especially if there is suboptimal response to antibiotics. There is need for the anticipatory education of health care professionals, who then can counsel parents and caregivers of young children to avoid oil/ghee administration and hence to avoid this easily preventable but potentially life threatening condition of lipoid pneumonia. Moreover, the involvement of media and other health agencies can also be rewarding

Distraction osteogenesis of a unilateral hypoplastic mandible

Distraction osteogenesis [DO] is a surgical process in which two bony segments are gradually separated so new soft tissue and bone will form between them by applying tension through a fixation device. There are three phases to this process: latency phase, distraction phase, and consolidation phase. The technique was originally applied to long bones but in recent years the method has been adapted for use in maxillofacial surgery. Distraction osteogenesis is a new variation of more traditional; orthognathic surgical procedures which can be applied for the correction of dento-facial deformities and syndromes of the jaws, treating upper airway obstruction in paediatric patients with mandibualr retrognathia, due to tongue collapse and physical obstruction in the hypopharangeal region. It is an effective and powerful reconstructive surgical technique, which can be performed safely without the need of bone graft or blood transfusion. We present treatment of a child with severe facial asymmetry after unilateral TMJ ankylosis corrected by distraction osteogenesis