The role of metabolic diseases in neonatal convulsions.

OBJECTIVE: The neonatal period is the most vulnerable time for the development of seizures, particularly in the first weeks after birth. These seizures often signify serious malfunction or damage to the immature brain and constitute a neurological emergency, necessitating urgent diagnosis and management. This study was performed to identify the etiology of convulsions during the neonatal period and to determine the rate of congenital metabolic disease. PATIENTS AND METHODS: A total of 107 term and preterm infants 0-28 days old who were treated and followed-up in the neonatal intensive care unit of our hospital between January 2014 and December 2019 were analyzed retrospectively based on data obtained by scanning the hospital information system and patient files. RESULTS: The study population included 54.2% male infants, and 35.5% of infants were born by caesarean section. Birth weight was 3,016 ± 560 (1,300-4,250) g, mean length of gestation was 38 (29-41) weeks, and mean maternal age was 27.4 ± 6.1 (16-42) years. Of the infants, 26 (24.3%) were preterm and 81 (75.7%) were term deliveries. Examination of family history revealed 21 (19.6%) cases with consanguineous parents and 14 (13.1%) cases with a family history of epilepsy. Hypoxic ischemic encephalopathy was the most common etiology of the seizures (34.5%). Burst suppression was detected on amplitude integrated electroencephalography in 21 (56.7%) monitored cases. Although subtle convulsions were most common, myoclonic, clonic, tonic and unclassified convulsions were also observed. The convulsions appeared during the first week of life in 66.3% of cases and during the second week or later in 33.7%. Fourteen (13.1%) patients examined by metabolic screening due to suspected congenital metabolic disease had a different congenital metabolic diagnosis. CONCLUSIONS: Although hypoxic ischemic encephalopathy was the most common cause of neonatal convulsions in our study, congenital metabolic diseases with autosomal recessive inheritance were detected at a high rate.

A new technique for metacarpophalangeal joint replantation after four-finger amputation.

Although finger amputations are not life-threatening, they are functionally and psychologically problematic for the patient. Successful replantation of amputated fingers results in better appearance and functional outcome. Therefore, the patient's satisfaction and psychological status improve. Usually, the first step of replantation involves bone and joint fixation. Various fixation methods can be used. Rapid and rigid fixation allows the surgeon to focus on repairing the soft tissues. In this report, we describe four-finger metacarpophalangeal (MCP) joint replantation with a new technique where no fixation device is used for the MCP joints. All four fingers survived and the total active motion of the four fingers ranged from 220 to 250 degree. Grip strength was 40kg and pinch strength was 5kg. Two-point discrimination was ranged from 4 to 8mm. We achieved a successful outcome comparable to previous reports. In conclusion, when amputation occurs at the MCP joint level, the fixation stage can be omitted to allow early repair of soft tissues.

An extraanatomic bypass method alternative to the obturator bypass.

Obturator bypass is the preferred method when infectious groin problems are challenging. However, this method can not be applied either in some special settings including the dissemination of the infection towards retroperitoneum and some technical difficulties in handling obturator bypass area (like fibrosis and inability to expose the obturator foramen). We present an alternative bypass method in two cases.

The primitive neuroectodermal tumor of the heart.

A young man was admitted to hospital with dyspnea, malaise, chest pain and night sweating. Investigative studies revealed a cystic mass lesion originating from the heart. Surgical exploration of the tumor showed that it was unresectable and pathology of the biopsy material was primitive neuroectodermal tumor. Medical literature concerning this unusual type of tumor is reviewed.

The Role of Polytetrafluoroethylene Graft Material in Buerger's Disease

The early and long-term results of polytetrafluoroethylene (PTFE) grafts in different anatomical locations were evaluated in 14 patients with Buerger's disease (thromboangiitis obliterans). During a 10-year period, 2160 patients with peripheral vascular disease were treated and, in 258, the diagnosis of Buerger's disease was made. Fourteen of these 258 patients underwent revascularization with PTFE grafts. In a follow-up period, up to 8 years patency rates were as follows: aorto/ilofemoral bypass 80%, femoropopliteal bypass 40%, femorocrural bypass 50% with a cumulative patency rate of 57.1%, and limb salvage rate of 88.9%. Because of the diverse nature and small number of the operations, the numerical results are not amenable to statistical analysis, but 88.9% limb salvage rate is satisfying in Buerger's disease. To the best of our knowledge, this is the first report on the use of PTFE grafts in Buerger's disease and it validates limb salvage even below the inguinal ligament.

A case of rapidly progressive pulmonary aneurysm as a rare complication of Behçet's syndrome--a case report.

One of the severe complications of Behçet's syndrome is pulmonary arterial involvement. The authors report a patient with Behçet's syndrome who had the complications of multiple pulmonary arterial aneurysms presenting with hemoptysis and right ventricular thrombus. He underwent emergency surgery because of failure of intensive immunotherapy and died 2 months after the surgery.

Management of aneurysms in Behçet's syndrome: an analysis of 24 patients.

BACKGROUND: The surgical therapy of Behçet aneurysms is often unsuccessful, resulting in graft occlusions, anastomoses, and/or new aneurysms. METHODS: Twenty-nine aneurysms were documented in 24 Behçet's patients during a period of 19 years. All patients were male, ranging in age from 20 to 53 years (mean, 35 +/- 7.3 years). The mean duration of disease was 9 +/- 5 years. There were nine abdominal aorta, four iliac, three common femoral, five superficial femoral, four popliteal, one subclavian, one carotid, and one posterior tibial artery aneurysm. In addition, in one patient an aneurysm developed from the arterialized venous conduit that had been inserted for a common femoral artery aneurysm elsewhere. Five patients were already under immunosuppressive therapy for ocular problems at the time of diagnosis. Fifteen patients received immunosuppressive therapy after operation. We performed one abdominal aneurysmorrhaphy, two iliac artery PTFE graft interpositions, two aortobiliac bypasses (PTFE), six aortic tube graft (three PTFE, three Dacron) interpositions, one avrtofemoral bypass (PTFE), two iliofemoral bypasses (PTFE), two superficial femoral artery graft (PTFE) interpositions, and three popliteal graft interpositions (one PTFE, two vein graft). Also as an initial procedure one carotid, one subclavian, four superficial femoral, one popliteal, and one posterior tibial artery were ligated. RESULTS: Nineteen patients were followed up for a mean duration of 47.3 +/- 27 months (range, 1 to 108 months). The patient with a subclavian aneurysm died of massive bleeding on postoperative day 15. Four patients were lost to follow-up. In the abdominal aortic aneurysm group one patient died of gastrointestinal bleeding 4 years after the operation. Another patient from the same group died 5 years after operation without any vascular disease. In the common femoral artery group the patient with an occluded iliofemoral graft died of an exsanguinating pulmonary artery aneurysm in the first year after operation. Overall, there were five anastomotic aneurysms. In addition, after the initial operation two iliofemoral, one aortofemoral, and one popliteal interposition graft were occluded without disabling ischemia. CONCLUSIONS: Aneurysms limited to the extremities could be ligated without disabling ischemia. Abdominal aortic aneurysms could be treated with tube graft insertion, giving satisfactory results. Patients could tolerate graft occlusion without major ischemia.

Surgical therapy of pulmonary arterial aneurysms in Behçet's syndrome.

Two patients with Behçet's syndrome were operated for exsanguinating pulmonary artery aneurysm into a bronchus. Lower lobectomy was performed in the first patient. He died of hypoxia and sepsis on the 8th postoperative day. Pneumonectomy was undertaken in the second patient, who is well 30 months after the operation.

Behçet disease: pulmonary arterial involvement in 15 cases.

PURPOSE: To describe findings at radiologic evaluation of pulmonary arterial involvement in Behçet disease. MATERIALS AND METHODS: Between 1978 and 1993, 31 pulmonary arterial aneurysms were diagnosed in 15 consecutive male patients, aged 21-56 years (mean, 32 years 5 months), with known Behçet disease. The patients were assessed at chest radiography (n = 15), computed tomography (n = 14), angiography (n = 10), and magnetic resonance (MR) imaging (n = 2). RESULTS: Three patients had single and 12 had multiple pulmonary aneurysms. In six patients, aneurysms were associated with pulmonary artery occlusion. Follow-up chest radiography revealed five subjects with progression leading to fatal rupture of aneurysms and two subjects with regression of the aneurysms and cessation of their symptoms. CONCLUSION: Intravenous digital subtraction angiography was the procedure of choice in the documentation of pulmonary arterial involvement, and MR imaging was most helpful in the presumptive diagnosis of occluded aneurysms. The appearance of pulmonary aneurysms represents a poor prognosis in patients with Behçet disease, but there is a chance that the aneurysms will not rupture.

Pulmonary arterial aneurysms in Behçet's syndrome: a report of 24 cases.

Pulmonary arterial involvement is an important complication of Behçet's syndrome (BS). Among 2179 patients with BS, 24 (1.1%) were diagnosed as having pulmonary arterial aneurysms (PAAs). Haemoptysis was the presenting symptom in all but one. All were male. The mean age at the time of the diagnosis of PAA was 30 +/- 11 S.D. yr (range 17-59 yr). Their mean disease duration was 5 +/- 4 yr (range 3 months-16 yr). There was a high prevalence of thrombophlebitis (21/24, 88%). Histopathological examination showed pulmonary vasculitis involving all layers of pulmonary arteries and veins. Twelve patients (50%) died after a mean of 9.5 +/- 11 S.D. months (range 1-36 months) after the onset of haemoptysis. The mean duration of follow-up of the remaining 12 patients was 25.5 +/- 24 S.D. months (range 1-78 months). The treatment consisted mainly of pulsed or oral cyclophosphamide alone or with prednisolone. As is true with other severe manifestations of Behçet's syndrome, PAAs are more common among males. They are associated with a prevalence of thrombophlebitis and there is high mortality despite treatment.

Behçet's disease presenting with a pulmonary mass lesion.

We report a case of Behçet's disease with pulmonary and splenic involvement. The radiologic presentation was quite different from the modes defined in the literature. A large, anteriorly located, well circumscribed ovoid mass was the x-ray film image of a large area of infarction with hemorrhage. The involved pulmonary artery showed signs of vasculitis with thrombus formation. To our knowledge, this mode of presentation is different from the cases reported in the literature.

Surgical treatment of Buerger's disease: experience with 216 patients.

Buerger's disease (thromboangiitis obliterans) is a rare peripheral vascular disease that usually affects young male smokers. It is characterized by multiple occlusions distal to the knee and elbow. Although rarely encountered in this condition, major arterial occlusions can be corrected by arterial revascularization. The hospital records of 216 patients (214 men and 2 women) with Buerger's disease were reviewed retrospectively, 142 (66%) between 1 and 120 (mean(s.d.) 72.8(24)) months after diagnosis. Of these patients 21 had had an arterial revascularization; 183 lumbar and 20 thoracic sympathectomies had been performed. In 30 of the patients a total of 29 minor and four major amputations had been performed. In appropriate cases arterial reconstruction provides better healing of ischaemic lesions. Although vasomotor tone is usually normalized in 2 weeks to 6 months after sympathectomy, the temporary increase in blood flow is often sufficient to heal ischaemic lesions during this period.

Peripheral aneurysms in Behçet's disease.

In this report, 14 patients with Behçet's disease with a total of 16 aneurysms are presented. The most common site of aneurysm formation was the femoral artery. Patients with Behçet's disease and aneurysm formation carry the highest morbidity and mortality rates among all those with the disease. The results of reconstructive surgery in the treatment of aneurysms are discouraging, with many anastomotic aneurysms and grafts occluding. In this study, it was possible to ligate major arteries such as the common iliac, superficial femoral and popliteal without limb loss. It is suggested that ligature either at initial surgery or reoperation should be performed to avoid complications of reconstructive surgery. Adjuvant immunotherapy is required to control relapses responsible for new aneurysm formation.