Normalization of serum lactic dehydrogenase in β-thalassemia patients following bone marrow transplantation

Serum lactic dehydrogenase (LDH) levels are mildly elevated in β-thalassemia major due to ineffective erythropoiesis. We reviewed the charts of 15 consecutive thalassemic children who underwent allogeneic, T-cell-depleted bone marrow transplantation (BMT) in our department during the last 3 years. Eleven patients had successful engraftment and are alive and well without evidence of disease, according to physical examinations, blood counts, and polymerase chain reaction (PCR) tests, with a median follow-up of 2 years. Two patients died due to transplantation-related complications, and two rejected the graft and received their backup autologous marrow. The LDH levels in the transplanted patients gradually decreased from an average of 952 ± 155 IU/L 10 days pre-transplant (N = 300–620) to 426 ± 56 IU/L at the day of transplantation, and stayed at approximately the same level post-transplant (489 ± 55 IU/L). By contrast, the LDH levels reverted to the pre-transplant value in those patients who rejected their marrow. The significance of this clinical observation for the pathophysiologic mechanism of intramedullary hemolysis and ineffective erythropoiesis in β-thalassemia major is discussed. © 1996 Wiley-Liss, Inc.     

An unusual peripheral osteoma of the mandibular notch: a case report with computed tomography evaluation

An osteoma, a slow-growing benign osseous neoplasm of the bone, is characterized by a proliferation of osteoblasts that form bone trabeculae in the connective tissue stroma. It is an uncommon condition that generally occurs in the maxillofacial bones. This neoplasm may be clinically silent for years without any symptoms. It is usually diagnosed when it becomes enlarged and is incidentally observed during a radiological examination. The radiographic characteristic of the lesion is a well circumscribed round or oval radiopaque mass. In this paper, we present a case of an unusual localization of a peripheral osteoma incidentally detected during radiographic examination with subsequent three-dimensional computed tomography evaluation.     

Incidental findings of carotid artery stenosis detected by calcifications on panoramic radiographs: report of three cases

Atherosclerosis, a progressive inflammatory disorder, may lead to coronary artery disease or stroke. The prevalence of atherosclerosis associated with mortality and morbidity is very high in developed countries, and is the underlying cause of approximately 50% of all deaths in Western societies. Panoramic radiographs (PRs) are an indispensable diagnostic tool used by dental practitioners. In the general dental population, the prevalence of positive incidental carotid artery calcifications (CACs) is reported to range from 2 to 5%. Therefore, incidental findings of CAC on PRs taken during routine dental evaluation may be useful for detecting asymptomatic patients at risk of stroke and could provide life-saving information. In this case report, we present radiological findings and follow-up of three patients with severe atherosclerosis that was incidentally detected on PRs, and discuss the role and importance of PRs in the detection of CACs.     

Renal antioxidant status in rats with hypertension induced by N sup omega nitro-L-arginine methyl ester

Nitric oxide (NO) has a role in the etiopathogenesis of hypertension. Relaxation of vascular smooth muscles is failed when NO production is reduced leading to increased vascular peripheral resistance. N sup omega nitro-L-arginine methyl ester (L-NAME) is one of the inhibitors of NO production. The aim of this study was to investigate oxidant-antioxidant systems of renal tissue in rats with hypertension induced by L-NAME. Rats were divided into three groups: control group and study groups treated with 100 or 500 mg/l L-NAME in drinking water for 15 days. The activities of catalase (CAT), glutathione peroxidase (GSH-Px) and superoxide dismutase (SOD), and the levels of malondialdehyde (MDA) and NO were studied in the renal tissue after hypertension induction. Arterial blood pressure was increased in both L- NAME groups. CAT activity of 500-mg L-NAME group was higher than control. GSH-Px activity of 500-mg L-NAME group was decreased compared with 100-mg ones. NO level was lower in 500-mg L-NAME group than control. MDA levels in both L-NAME groups were decreased compared with control. In conclusion, hypertension was induced with oral L-NAME treatment. Increased CAT activity was compensated with decreased GSH-Px activity in 500-mg L-NAME group. Both study groups were protected from lipid peroxidation with NO inhibition.     

Hemimegalencephaly and Hirschsprung’s Disease: A Unique Association

A 2-year-old boy with hemimegalencephaly and Hirschsprung’s disease is reported. The unique association of these two entities is considered to be the presence of a common insult or insults that affect the innervation of the bowel and the formation of the cerebral cortex. Short-segment subtype of Hirschsprung’s disease may suggest that this effect occurred between the eighth and twelfth weeks of gestation. Although there is a well-known coexistence of Hirschsprung’s disease with the malformations that share a common neurocristopathic origin (abnormalities of neural crest cell growth, migration, or differentiation), a few extremely rare cases, as in this case, might reflect the coexistence of Hirschsprung’s disease with a cerebral malformation (i.e., hemimegalencephaly) that is a nonneurocristopathic entity by itself.     

Treatment of tripod fracture of zygomatic bone by <Emphasis Type="Italic">N</Emphasis>-2-butyl cyanoacrylate glue fixation,and its effects on the tissues

The standard treatment of zygomatic bone fractures is fixation by microplates or miniplates and screws today. It is very difficult to place plates and screws into thin bones or small bone fragments especially in the facial bones and bones adjacent to important structures. Cyanoacrylate is used as a hemostatic agent, an embolic agent, in retinal tears, in corneal ulcers, in fixation of mandibular osteotomies and in craniofacial surgery. N-2-Butyl cyanoacrylate is a form of cyanoacrylate which is bioabsorbable and biocompatible. It is easily applied to the tissues. We aimed to determine the effect of N-2-butyl cyanoacrylate in the fixation of displaced zygomatic bone fractures. We examined the histotoxicity and the effects on healing and foreign body reaction of N-2-butyl cyanoacrylate. Eight New Zealand white rabbits underwent zygomatic osteotomies bilaterally. The fractures on left sides of the rabbits were determined as study site and right sides as control site. Knight and North classification of zygomatic bone fractures were used and group 4 fractures were made bilaterally. Open reduction of fractures was performed bilaterally and N-2-butyl cyanoacrylate was applied only on left sides. No fixation was made on right sides representing the control group. Postoperatively in the first, second, third and fourth weeks, two rabbits were sacrificed and the fracture sites were examined macroscopically and histopathologically. In the glued study group, fixation was obtained in all cases whereas in the control group, all the fractures were seen to be displaced. Tissue reaction was similar in the study and the control groups.     

Deep brain stimulation for Holmes’ tremor related to a thalamic abscess

BACKGROUND: Medical treatment is usually ineffective for Holmes' tremor, and surgery is the treatment of choice for many patients. Here we report the case of a 14-year-old girl who developed Holmes' tremor related to a thalamic abscess and was successfully treated with thalamic deep brain stimulation. CASE REPORT: The patient presented with left hemiparesis and headache and was hospitalized. Investigation revealed a thalamic abscess in the left cerebral hemisphere. The abscess was drained via stereotactic surgery and a course of antibiotic treatment was completed. Four months after treatment, the patient developed Holmes' tremor in her left upper extremity. When attempts at medical treatment with levodopa, clonazepam, and trihexyphenidyl all failed, an implant was placed and deep brain stimulation of the ventral intermediate nucleus of the thalamus was initiated. During 2.5 years of follow-up, her tremor diminished by 90%. CONCLUSION: This case demonstrates that medically resistant Holmes' tremor related to a thalamic lesion can be successfully treated with thalamic deep brain stimulation.     

Prevention of brisk hyperactive response during selective dorsal rhizotomy in children with spasticity: Isoflurane versus sevoflurane maintenance anesthesia

In children with spasticity, deep tendon reflexes are hyperactive and even stimulation of normal dorsal rootlets can produce exaggerated full-strength, single-twitch responses in the muscles they innervate. This phenomenon is called the brisk hyperactive response (BHR). The aim of this study was to compare the efficacy of 2 volatile anesthetics, isoflurane and sevoflurane, for suppressing the confounding effect of BHR during selective dorsal rhizotomy (SDR) in children with spasticity. The subjects were 54 consecutive children of American Society of Anesthesiology physical status III who were scheduled for SDR. After tracheal intubation, each child was randomly assigned to Group I (isoflurane; n = 27) or Group S (sevoflurane; n = 27). There was no significant difference between the mean operation times in Groups I and S (200 ± 40 vs. 220 ± 35 minutes, respectively; p = 0.0559). Thirteen patients in Group I (48.1%) and 5 in Group S (18.5%) exhibited BHR during stimulation of the dorsal rootlets (odds ratio 4.086; p = 0.0418). Three of these 18 patients (2 in Group I and 1 in Group S) experienced hypertension and tachycardia simultaneously with BHR (odds ratio 4.086; p = 1.0). The results suggest that sevoflurane is more effective at preventing BHR and might be a better choice for anesthetic management of children with spasticity undergoing SDR.