Medium term outcome for infant repair in tetralogy of Fallot: indicators for timing of surgery

Objective: To assess the impact of early corrective surgery on the short and medium term outcome in tetralogy of Fallot (TOF). Materials and methods: All patients under 12 months of age undergoing correction of isolated TOF between February 1997 and July 2003 were reviewed retrospectively. Outcome data for mortality, post-operative care management, major morbidity and clinical follow-up were analysed. Results: Fifty-two operations were performed. The mean age at surgery was 5 months (range 1–12) of whom 16 (30.8%) were less than 3 months old, including 2 neonates, 22 (42.3%) were 3–6 months old and 14 (26.9%) were 7–12 months old. There was 1 (1.9%) early death caused by a cerebro-vascular accident and 1 (1.9%) late death secondary to acute infective endocarditis. There were no differences in post-operative morbidities attributable to age. Patients under 3 months old required greater duration of post-operative ventilation, ITU stay and in-hospital stay. At a mean follow-up of 4.0 years (range 1.5–8.0), 33 (63.5%) patients had well-tolerated pulmonary regurgitation (PR) and 3 (5.8%) patients required re-operation for right ventricular outflow tract obstruction (RVOTO). All patients had right bundle-branch-block but with QRS < 150 ms. Conclusion: Early definitive repair of TOF can be performed safely on patients under 6 months old. Age at surgery does not appear to affect the medium term haemodynamic outcome. However, early surgery does escalate the need for ICU care. This data suggests repair in asymptomatic patients be delayed until 3–6 months of age.     

Stimulation of nerve growth factor and substance P expression in the iris–trigeminal axis of diabetic rats—involvement of oxidative stress and effects of aldose reductase inhibition

In rats with streptozotocin-induced diabetes, we measured increased (by 61%; P<0.05) mRNA for nerve growth factor (NGF) in the iris together with increased (by 82%; P<0.05) mRNA for preprotachykinin (the substance P precursor) in the trigeminal ganglion, suggesting that increased NGF was driving increased substance P gene expression. In other diabetic rats, these changes were prevented by treatment with either an antioxidant (butylated hydroxytoluene; 1% by diet) or an aldose reductase inhibitor (ARI) (sorbinil; 25 mg/kg/day p.o.) and the sorbinil treatment was associated with significant inhibition of polyol pathway intermediates in both lens and sciatic nerve. This suggests that polyol pathway activity in the lens may translate to oxidative stress-driving stimulation of NGF gene expression in the iris. The change is selective for NGF, because expression of the analogous neurotrophin, neurotrophin-3 (NT-3), was unaltered in the same irises. These changes suggest that oxidative stress and/or inflammation can drive up NGF expression in diabetes—a mechanism that might participate in iritis.     

Laparoscopic bilateral adrenalectomy following failed hypophysectomy

Background: Laparoscopic adrenalectomy has recently been shown to be a safe and effective means of treating adrenal pathology with much lower morbidity than the traditional approach. The majority of reports in the literature involve removal of adrenal tumors. Although open bilateral adrenalectomy has been utilized for persistent Cushing's syndrome following attempted hypophysectomy, there is little data available describing the application of laparoscopic adrenal surgery to this problem. Methods: Four patients with persistent Cushing's syndrome after attempted treatment with hypophysectomy underwent laparoscopic bilateral adrenalectomy at our institution. One procedure was done transabdominally in the supine position. Three procedures were done transabdominally using sequential lateral decubitus positions. Results: All procedures were completed laparoscopically. The mean operative time was 4.6 h (range 3.9–5.25). Repositioning and reprepping the patients resulted in a slight increase in operative time, but visualization was improved using the lateral decubitus position. Average blood loss: 156 cc (range 50–300). One patient required early reoperation for bleeding from the left adrenal bed, which was controlled laparoscopically. Three patients were eating the following day and were discharged on postoperative days 1, 2, and 5. The fourth patient remained hospitalized for 18 days due to problems unrelated to surgery. After a mean follow-up of 10 months, all patients have done well and have no clinical or biochemical evidence of recurrent disease. Conclusion: Our clinical experience indicates that laparoscopic bilateral adrenalectomy is a viable treatment option for Cushing's syndrome following failed hypophysectomy. Received: 29 March 1996/Accepted: 12 June 1996     

Flat adenomas in a colon cancer-prone kindred

We describe new pathologic findings in a hereditary nonpolyposis colorectal cancer family. Affected family members developed multiple small adenomas with right-sided predominance; many adenomas had an unusual appearance featuring slightly elevated lesions with adenomatous changes confined to the upper regions of the colonic crypts. We have adopted the previously established term "flat adenoma" for these lesions. This phenotype may be a morphologic marker for at least one subset of hereditary nonpolyposis colorectal cancer.     

Infants at risk for schizophrenia: sequelae of a genetic neurointegrative defect. A review and replication analysis of pandysmaturation in the Jerusalem Infant Development Study.

A 1975 report stated that a schizophrenic genotype may be manifested in infants by a neurointegrative defect called pandysmaturation. Recent evidence supports this: (1) 12 studies found delayed development in schizophrenics' infants and in preschizophrenics; (2) "blind" psychometric evaluations favored an adult schizotypal disorder in four to six of seven high-risk subjects with pandysmaturation in the New York study; and (3) finally, in a partial replication of this method using the Jerusalem data, blind diagnoses of "probable" and "possible" pandysmaturation were significantly related to a parental diagnosis of schizophrenia and to cognitive and motor neurointegrative deficits at 10 years. Obstetrical complications were unrelated to diagnosis, pandysmaturation, or outcome in the overall sample. However, we found a small subgroup of schizophrenic offspring in whom the most severe motor deficits at follow-up were related to obstetrical complications, pandysmaturation, and low birth weight.     

Rehabilitation of the knee after anterior cruciate ligament reconstruction

From the Kerlan-Jobe Orthopaedic Clinic, 501 E. Hardy Street, Suite 200, Inglewood, CA 90301. New information regarding the isometric placement of the anterior cruciate ligament (ACL) substitute, revascularization process, and biomechanical stresses have all contributed to and been incorporated in the rehabilitation program after ACL reconstruction. Treatment protocols specifically designed for the patient following ACL reconstruction are imperative to return the individual to his or her preinjury status. Care is taken to limit the amount of stress placed on the ligament substitute especially at end of range extension. A program incorporating techniques for developing range of motion and strength while still preserving stability at the knee joint is still of the utmost importance. This program is a revision of a previously reported regimen from this facility (Brewster, Moynes, Jobe, J Orthop Sports Phys Ther 5:121-126, 1983) and is based upon clinical experience and research information. J Orthop Sports Phys Ther 1989;11(1):8-18.     

Role of mast cells in experimental anti-glomerular basement membrane glomerulonephritis

Recently, divergent reports on the role of mast cells (MC) in different glomerular diseases have brought our attention to their role in an accelerated model of anti-glomerular basement membrane (GBM) glomerulonephritis (GN). Genetically MC-deficient Kit(W)/Kit(W-v) mice, MC-reconstituted Kit(W)/Kit(W-v) mice and Kit+/+ control mice were subjected to anti-GBM GN. Kit(+/+) mice developed moderate proteinuria and glomerular damage following the induction of anti-GBM nephritis. In contrast, proteinuria and glomerular damage were dramatically increased in MC-deficient Kit(W)/Kit(W-v) mice. MC-reconstituted Kit(W)/Kit(W-v) mice showed proteinuria and glomerular damage comparable to Kit+/+ mice. A significant increase in infiltrating T cells and macrophages was detected in MC-deficient Kit(W)/Kit(W-v) mice as compared to Kit+/+ control mice and MC-reconstituted Kit(W)/Kit(W-v) mice. Accordingly, we observed an increase of TGF-beta1 mRNA in kidneys from Kit(W)/Kit(W-v) mice. Interestingly, we did not detect MC in the kidney using either Giemsa staining or RT-real-time PCR, but MC were found in the regional lymph nodes. Finally, mortality of Kit(W)/Kit(W-v) mice was significantly increased after the induction of anti-GBM GN due to uremia. Our report provides the first direct evidence that MC are protective in anti-GBM GN, possibly by modulating the influx of effector T cells and macrophages to inflammatory sites in the kidney.