Hasan Tahsin Keceligil

Eculizumab therapy in a patient with dense-deposit disease associated with partial lipodystropy.

BackgroundDense deposit disease (DDD) (also known as membranoproliferative glomerulonephritis type II) in childhood is a rare glomerulonephritis with frequent progression to end-stage renal disease (ESRD) and a high recurrence after kidney transplantation. The pathophysiologic basis of DDD is associated with the uncontrolled systemic activation of the alternative pathway (AP) of the complement cascade.Case-diagnosis/treatmentA 14-year-old girl presented with edema and nephrotic range proteinuria. Blood tests showed hypoalbuminemia, nephrotic range proteinuria, normal renal function, and a low C3 level. Renal biopsy confirmed the diagnosis of crescentic DDD. Complement analysis revealed strong AP activation (low C3), positive C3 nephritic factor (C3NeF), and a decreased complement factor H (CFH) levels with CFH polymorphisms. Therapy with eculizumab was considered after the failure of corticosteroid and plasmapheresis to modulate the ongoing massive proteinuria and persistence of low serum C3 levels. There was a marked clinical and biochemical response following the administration of eculizumab.ConclusionsOur case emphasizes the efficacy of eculizumab in the management of crescentic DDD in a patient with a normal renal function, in a short follow-up period. Considering previously reported cases, it appears that eculizumab represents a promising new approach which may prevent progression to ESRD in a subset of patients with DDD.

Tracheoinnominate artery fistula following tracheostomy.

Tracheoinnominate artery fistula is a relatively rare but highly lethal complication occurring in patients with long-standing tracheostomies. Early evaluation of this problem and prompt aggressive therapy are necessary. When massive haemorrhage begins, immediate arterial compression, control of the airway and subsequent treatment of the injured artery may be lifesaving. Immediate surgical exploration through a median sternotomy is necessary to control the proximal and distal innominate artery. After the damaged artery has been excised, vascular reconstruction can be performed to preserve the connection between the proximal and distal ends of the innominate artery. A pedicled pericardial patch was successfully used for the tracheal reconstruction.

Primary Surgical Repair of Ventricular Septal Defect

Between January 1983 and December 2000, 78 patients underwent primary repair of a ventricular septal defect. There were 42 males (54%) and 36 females (46%) of whom 13 (17%) were under 1 year old, 50 (64%) were aged 1–10 years, 11 (14%) were aged 10–20 years, and 4 (5%) were over 20 years old. The ventricular septal defect was a perimembranous type in 60 patients (77%), subarterial (outlet) type in 10 (13%), and atrioventricular canal (inlet) type in 4 (5%). Operative repair was performed with a patch in all except 2 patients. Early postoperative complications included insignificant aortic regurgitation in 4 patients, persistent complete heart block in 1, and residual shunt in 4. There were 5 early deaths (6.4%) and 1 late death (1.8%) in 56 patients followed up. Early primary closure of ventricular septal defects, usually via a right atriotomy, can be performed with acceptable mortality and morbidity rates.

Thyroid hormone alterations during and after cardiopulmonary bypass

The haemodynamic effects of thyroid hormones are well known and include those on heart rate, contractility and myocardial oxygen consumption. Cardiopulmonary bypass produces various alterations in endocrine homoeostasis and may exert important haemodynamic effects postoperatively. The purpose of this prospective study was to determine the relation of cardiopulmonary bypass to changes in thyroid function. Blood samples were obtained from 20 patients preoperatively, at specific times before, during and after cardiopulmonary bypass. Total thyroxin (TT4), total triiodothyronine (TT3), free thyroxin (fT4), free triiodothyronine (fT3), thyroid-stimulating hormone (TSH), thyroid-binding globulin (TBG) and albumin were measured by radioimmunoassay and competitive immunoassay. Values of TT4, TT3 and fT3 were significantly depressed up to 24 h after cardiopulmonary bypass (P < 0.05). TSH and fT4 levels remained within normal ranges at all sampling times. It is increasingly evident that cardiopulmonary bypass affects thyroid hormone metabolism, leading to a transient depression characterized by low levels of circulating TT4, TT3 and fT3.

A Forgotten Guidewire Causing Intracardiac Multiple Thrombi With Paradoxical and Pulmonary Embolism

A woman aged 70 years was transferred from another medical centre to our hospital because of shortness of breath, hemoptysis, and ischemic changes in the right foot (Fig. 1A). She had chronic renal failure and had been enrolled in a hemodialysis treatment program for 6 years. Transesophageal echocardiography showed a guidewire in the right atrium and right ventricle, with multiple mobile thrombi attached to it. The guidewire entered the right atrium through the superior vena cava, passed into the right ventricle through the lateral tricuspid valve annulus, and then looped in the right ventricle. One of the thrombi was entrapped within a patent foramen ovale and oscillated between right and left atria (Fig. 1B; Video 1 ; view video online). Pulmonary computed tomography angiography showed emboli in the segmental artery of the right lower lobe and a subsegmental artery of the left lower lobe. Because of the precarious state of the patient and the risk of further emboli, it was decided to take the patient for emergency cardiac surgery. The intracardiac guidewire and multiple thrombi were removed successfully (Fig. 1C). Unfortunately, because of multiple comorbidities, hemodynamic instability, and profound acidosis, she died on the second postoperative day. Vascular interventional procedures are widely used, resulting in increased complication rates. Guidewire loss may lead to thrombosis, embolism, sepsis, arrhythmia, or perforation. Loss of a complete guidewire is a rarely reported complication. Percutaneous retrieval is the preferred treatment, with low complication and high success rates. Various devices and techniques, such as loop snares, 2-wire or triplewire technique, basket retrievers, special catheters, grasping

Inhibition of vein graft intimal hyperplasia by periadventitial application of hyaluronic acid–carboxymethyl cellulose: An experimental study

Objective. Placement of an external support has been reported to prevent intimal hyperplasia of vein grafts. In this study, we investigated the effect of HA/CMC on intimal hyperplasia in a rabbit model. Design. Right jugular vein to common carotid artery bypass grafting was performed in 24 female New Zealand white rabbits (2.5–3.0 kg). Animals were divided into two groups: control group (n=12) and HA/CMC group (n=12). Absorbable membrane barrier was wrapped around vein grafts in HA/CMC group. In control group, no material was applied following venous graft bypass. Results. At 1 month, in the vein grafts supported with the HA/CMC membrane neointimal thickening was significantly less (109 µm [IQR, 78–166]) compared to the unsupported control grafts (220 µm [IQR; 101–312]; p<0.001). Medial thickening in the HA/CMC group (128 µm [IQR, 101–181]) compared to unsheathed control grafts (182 µm [IQR, 131–255] p<0.001) was also significantly less. Conclusion. Periadventitial placement of HA/CMC as an absorbable membrane inhibits intimal hyperplasia of vein bypass grafts in a rabbit model.

Giant extracranial internal carotid artery aneurysm.

77-year-old woman, with a history of hypertension, Adyslipidemia, and vitiligo was admitted to our hospital with a large growing palpable mass in the right side of the neck. She had symptoms of pain in the right ear, headache, dizziness, dysphagia, and hoarseness. Physical examination revealed a large pulsatile mass in the right cervical region. Electrocardiography showed a sinus rhythm with a heart rate of 80 beats/min. Transthoracic echocardiography showed normal left ventricular systolic

Successful combination of interventional and surgical treatment of left atrium perforation

Percutaneous interventions are increasingly used in the treatment of cardiac diseases which are resistant to medical treatment. However, the complications caused by these interventions can lead to serious results. In this article, we present a case of a successful combination of interventional and surgical treatment methods, following the development of left atrial perforation during radiofrequency catheter ablation (RFA), in a patient with atrial fibrillation resistant to medical treatment.