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91.
Alkaptonuria is a metabolic disorder in which homogentisic acid oxidase is absent. Therefore, homogentisic acid accumulates in cartilage and connective tissues. We can diagnose ochronotic arthropathy, a manifestation of long standing alkaptonuria, through careful radiological, physical, and laboratory examination. In this report, we describe 4 cases of ochronotic arthropathy to which we applied cementless total hip prosthesis due to severe hip involvement.  相似文献   
92.
BACKGROUND: The management of the apical multiple muscular ventricular septal defects (VSDs) remains still controversial. There are various surgical techniques and approaches for closure of "Swiss-cheese" VSDs. In this study, we report the outcome of multiple muscular VSDs repair, using the septal obliteration technique. METHODS: We used the septal obliteration technique in five "Swiss-cheese" ventricular septal defects cases through right atriotomy. Four of the cases had isolated multiple VSDs. One case also had an aortic arch interruption type A, which was repaired prior to cardiopulmonary bypass. Their ages varied between 43 days and 6 years. RESULTS: We did not experience any mortality or serious morbidity. Tracheostomy was required in one patient. There was no important residual shunt in postoperative period, except a minimal shunt in one case. CONCLUSION: The closure technique of "Swiss-cheese" trabecular multiple VSDs using a large single patch was not troubling. Transatrial approach prevented postoperative problems of ventricular incision. Using a large needle with a large pledgett is the key, which provides deep tissue penetration to avoid residual shunt.  相似文献   
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OBJECTIVE: To investigate whether an interaction exists between nocturnal enuresis and allergy. MATERIAL AND METHODS: Thirty-seven (20 boys, 17 girls) children with monosymptomatic nocturnal enuresis were recruited. We studied an allergy panel that included total IgE, 10 examples of inhalant-specific IgE, 10 examples of food-specific IgE, eosinophilic cationic protein (ECP) and Phadiotop. The same panel was studied in a control group of 18 children without monosymptomatic nocturnal enuresis. RESULTS: We did not determine statistically significant differences between the enuretic group and the control group in terms of levels of total IgE, the 10 examples of inhalant-specific IgE and Phadiotop. However, two (soybean and hazelnut) of the 10 food-specific IgE and ECP levels did differ significantly between the two groups. CONCLUSIONS: This first specific IgE study showed that there may be a relationship between nocturnal enuresis and soybean and hazelnut food allergens. Our findings may explain some cases of nocturnal enuresis. However, further studies are necessary to explain the underlying mechanisms and management of this disorder.  相似文献   
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BACKGROUND: Renin-angiotensin system (RAS) was suggested to modulate inflammatory cytokine production. Angiotensin II was consistently shown to increase production of tumor necrosis factor alpha (TNF-alpha). However, inflammatory cytokines and RAS were modulated by genetic polymorphisms such as TNF-alpha-308 G > A and angiotensin-converting enzyme (ACE) I/D gene polymorphisms. The aim of this study was to investigate the effects of ACE and TNF-alpha genotypes on inflammatory cytokines in hemodialysis (HD) patients. METHODS: ACE I/D and TNF-alpha-308 G > A genotypes, pre- and postdialysis plasma renin activity (PRA), serum ACE, interleukin-1 beta (IL-1beta), and TNF-alpha levels were determined in 22 HD patients. RESULTS: Predialysis serum ACE activity is correlated with TNF-alpha (r = 0.63; P = 0.01), and PRA was correlated with IL-1beta levels (r = 0.49; P = 0.02). Pre/postdialysis IL-1beta and TNF-alpha were similar in DD and II/ID ACE genotypes. Predialysis TNF-alpha and IL-1beta (32.4 +/- 5; 35.1 +/- 4.2 vs. 28.1 +/- 3.7; 26.5 +/- 6.2 pg/mL; P < 0.05) and postdialysis TNF-alpha levels (30.4 +/- 1.4 vs. 28.4 +/- 0.82 pg/mL; P < 0.05) were significantly higher in TNF1/2 than TNF1/1 patients. CONCLUSION: ACE and TNF-alpha-308 G > A (1/2) gene polymorphisms may contribute to modulation of proinflammatory cytokine production and hence chronic inflammation in HD patients.  相似文献   
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The purpose of this prospective study was to evaluate microsurgical thyroidectomy by comparing it with traditional thyroidectomy. Before surgery, patients were assigned either to the microscopic thyroidectomy group (MT group), with the use of the surgical microscope, or the traditional thyroidectomy group (TT group), without the use of visual magnification. Outcome measures were operative time, intraoperative bleeding and complication rates including injury to the recurrent laryngeal nerve (RLN), the external branch of the superior laryngeal nerve (EBSLN) or the parathyroid glands. Ninety-eight patients underwent thyroid surgery (58 patients in the MT group, 40 patients in the TT group). The two groups were similar in age, sex, surgical procedures and histological findings. There was no difference between the two techniques regarding the operative time and the amount of blood loss. Neither permanent nerve palsy nor persistent hypocalcemia occurred in either group. Transient nerve palsies (RLN and EBSLN) were lower in the MT group (1.7%) compared to the TT group (7.5%), but the difference did not reach statistical significance (P>0.05). Overall transient hypocalcemia was significantly lower in the MT group (1.7%) compared with the TT group (12.5%, P=0.032). If the population was restricted to total thyroidectomy, the rate of transient hypocalcemia was 4.1% in the MT group and 33.3% in the TT group, respectively (P=0.022). In conclusion, microsurgical thyroidectomy is a feasible and efficacious surgical procedure. It significantly reduces the complications without increasing the operating time in thyroid surgery procedures. A major advantage of this technique is the possibility of attaching a camera to the microscope, thereby greatly facilitating teaching.The authors did not receive any financial support for this study.  相似文献   
99.
SUMMARY: The aim of this study was to evaluate the psychiatric symptoms among adolescents who were seen in the outpatient clinic for their physical complaints. Two hundred and ninety adolescent outpatients (154 males and 136 females) between 13 and 17 years of age (mean 14.3+/-1.2) are included in this study. Patients with known psychiatric disorders, mental retardation, organic brain diseases, or chronic organic problems were excluded. The Brief Symptom Inventory, which measures the psychiatric symptoms under the categories of anxiety, depression, negative self, somatization, and hostility, was given to all subjects. Symptoms of urinary and cardiovascular systems were related to hostility. Patients with obesity, hirsutism, problems of external genitalia, enuresis nocturna, abdominal pain, chest pain, and lack of weight gain showed psychiatric symptoms at pathological levels. Hostility symptom was found to be high in all groups.  相似文献   
100.
A unique case of bilateral severe carpal tunnel syndrome due to familial Mediterranean fever is reported. The syndrome was diagnosed by clinical examination and electrophysiological studies. Bilateral transverse carpal ligaments were released and the biopsy specimens revealed systemic type A amyloidosis. Up to our knowledge, the co-existence of bilateral carpal tunnel syndrome and familial Mediterranean fever has not been reported previously in the literature.  相似文献   
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