Partial tarsal arthrodesis for rheumatoid feet

Partial intertarsal joint arthrodesis was performed on 12 feet of 11 patients as a surgical treatment for planovalgus deformity of the foot and lesions of the intertarsal joints caused by rheumatoid arthritis. Single arthrodesis was performed on the talocalcaneal joint in eight feet, two of which underwent simultaneous total ankle arthroplasty, and on the talonavicular joint in two feet. Double arthrodesis was done on the talocalcaneal and talonavicular joints in one foot and on the talonavicular and calcaneocuboid joints in one foot. Screws or staples were used for fixation. Patients were followed for 2 years to 8 years 7 months (average 4 years 3 months). Osseous fusion was achieved in all feet, and satisfactory pain relief was obtained in all cases except one. We performed this surgery in patients who were relatively active, and the results indicated that arthrodesis of a small number of joints that caused pain and deformity was effective in reducing pain and correcting the deformity. We concluded that partial tarsal arthrodesis should be performed on a limited number of joints during the early stages of planovalgus deformity of the foot because more joints are found to be fixed during the advanced stages. However, progression of the osteoarthritis was found in the neighboring joints. Close follow-up observation is needed.     

Identical twins with long QT syndrome associated with a missense mutation in the S4 region of the HERG

Familial long QT syndrome (LQTS) is caused by mutations in genes encoding ion channels important in determining ventricular repolarization. Mutations in at least five genes have been associated with the LQTS. Fire genes, KCNQ1, HERG, SCN5A, KCNE1, and KCNE2, have been identified. We have identified a missense mutation in the HERG gene in identical twins in a Japanese family with LQTS. The identical twins in our study had QT prolongation and the same missense mutation. However only the proband had a history of syncope. Although many mutations in LQT genes have been reported, there are few reports of twins with LQTS. This is the first report, to our knowledge, of identical twins with a HERG gene mutation.     

Four types of Ipsilateral Breast Tumor Recurrence (IBTR) after breast‐conserving surgery: Classification of IBTR based on precise pathological examination

We classified ipsilateral breast tumor recurrences (IBTRs) based on strict pathological rules. Ninety‐six women who were surgically treated for IBTR were included. IBTRs were classified according to their origins and were distinguished based on strict pathological rules: relationship between the IBTR and the primary lumpectomy scar, surgical margin status of the primary cancer, and the presence of in situ lesions of IBTR. The prognosis of these subgroups were compared to that of new primary tumors (NP) in the narrow sense (NPn) that occurred far from the scar. Distant‐disease free survival of IBTR that occurred close to the scar with in situ lesions and a negative surgical margin of the primary cancer (NP occurred close to the scar, NPcs) was similar to that of NPn. In contrast, IBTR that occurred close to the scar without in situ lesions (true recurrence (TR) that arose from residual invasive carcinoma foci, TRinv) had significantly poorer prognosis than NPn. IBTR that occurred close to the scar with in situ lesions and a positive surgical margin of the primary cancer (TR arising from a residual in situ lesion, TRis) had more late recurrences than NPcs. Precise pathological examinations indicated four distinct IBTR subtypes with different characteristics.     

Cutaneous Mycobacterium chelonae infection following autologous peripheral blood stem cell transplantation for POEMS syndrome

Although hematopoietic stem cell transplantation (HSCT) may increase the curability of refractory hematologic diseases, it requires complication management due to a long-term immunocompromised state. We experienced a case who received an autologous peripheral blood stem cell transplantation (Auto-PBSCT) for POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) and developed cutaneous Mycobacterium chelonae infection. It is clear that attention needs to be paid to prevent bacterial, fungal and viral infection after HSCT. It is also important to keep in mind that tuberculous and nontuberculous mycobacteria (NTM), in rare cases, lead to lethal complications.     

The role of leukocyte depletion by in vivo use of leukocyte filter in lung preservation after warm ischemia

The oxygen metabolites of leukocytes have been implicated as playing the central role in reperfusion injury of preserved lung for transplantation. Furthermore, it is still unknown how leukotrienes take part in this process. Twelve mongrel dogs were used, divided into two groups. In group A, left thoracotomy and hilar stripping of the left lung was performed under anesthesia. During warm ischemia (two hours) and reperfusion (two hours), a filter was used three times between the femoral artery and vein, and quantitative in vivo blood filtration was performed. In group B, warm ischemia and reperfusion was done in the same fashion without filtration. As for tissue free radical assay, lipid peroxidation products were measured by means of thiobarbituric acid. Tissue leukotriene B4 and C4 levels were measured by radio immunoassay. In addition, peripheral blood count and tissue wet/dry ratio were analyzed. Significant leukocyte depletion of peripheral blood with leukocyte efflux from reperfused lung was observed in group A. Significant increase of tissue malondialdehyde (MDA) titers after reperfusion was observed in group B, whereas no significant fluctuation of tissue MDA titers was noted in group A. Concerning leukotriene B4 and C4, no significant increase was observed in either group. Tissue damage estimated by lung wet/dry ratio was considered to be controlled in group A compared with group B. Thus, the authors conclude that in vivo use of a leukocyte filter is efficient in removal of peripheral blood leukocytes and in preservation of reperfused lung after warm ischemia and that oxygen metabolites of leukocytes are considered to play a role in tissue damage in this process.     

Angiotensin II plays an important role in maintaining blood pressure in postmenopausal women receiving hormone replacement therapy

BACKGROUND: We investigated the role of angiotensin (Ang) II in maintaining blood pressure (BP) by administering a small dose of candesartan, an Ang II type 1 receptor antagonist, in postmenopausal women receiving long-term hormone replacement therapy (HRT). METHODS: A single dose of 2 mg of candesartan was administered orally to 13 normotensive postmenopausal women receiving HRT (continuous combined conjugated estrogen and medroxyprogesterone acetate orally; HRT group) and 13 normotensive postmenopausal women not receiving HRT (control group). Both BP and heart rate (HR) were measured at baseline and at 1, 2, 3, 4, 5, and 6 h after administration. Plasma renin activity (PRA) and Ang I, Ang II, and bradykinin concentrations were measured at baseline and 4 h after the administration of candesartan. RESULTS: Candesartan lowered the BP and raised the HR in both groups. However, the decrease in BP was significantly greater in the HRT group than in the control group (P < .05), whereas no significant difference in the change in HR was observed between the two groups. In the HRT group, significant increases were found in PRA, Ang I, and Ang II (all P < .05) and a significant decrease in bradykinin (P < .01) with candesartan treatment. In the control group, candesartan as associated with an increase in PRA (P < .05) but not in Ang I, Ang II, or bradykinin. CONCLUSIONS: Based on our study results, Ang II plays an important role in maintaining BP in normotensive postmenopausal women receiving HRT. Maintenance of BP may be dependent on the balance between the hypertensive effect of Ang II and the hypotensive effect of bradykinin.     

Rare association of tetralogy of Fallot and aortic valve stenosis. Autopsy findings after failed balloon aortic valvuloplasty.

We report the case of a neonate with a rare association of tetralogy of Fallot and aortic valve stenosis, who died following percutaneous balloon valvuloplasty. Postmortem examination confirmed a tetralogy of Fallot morphology with a markedly hypoplastic pulmonary tree, hypertrophy of both ventricles, and a thickened and dysplastic aortic valve. Valvuloplasty was not effective despite the presence of a fissure in the cusp. The findings suggest that the left ventricular impairment and associated anomalies, with or without a dysplastic valve may contribute to the failure of balloon valvuloplasty in neonates with critical aortic valve stenosis.