Congenital hepatoportal arteriovenous fistula: Report of a case

(Received for publication on Nov. 20, 1998; accepted on Sept. 17, 1999)     

Arterial remodelling in Fabry disease

Aim : The enzymatic defect in Fabry disease results in the slow systemic deposition of uncleaved glycosphingolipids in the lysosomes of vascular endothelium and smooth muscle cells, leading to ischaemic strokes, cardiomyopathy and renal failure. Whereas it is known that Fabry disease affects small blood vessels, little is known about its effects on peripheral large arteries. We therefore set out to compare parameters of arterial wall structure and function in a cohort of patients with Fabry disease and an age-matched control group. Methods : Large artery phenotype was non-invasively investigated in 21 hemizygous patients with Fabry disease and 24 age-matched male controls. Common carotid and radial artery diameter, intima-media thickness (IMT) and distensibility were determined with high-definition echotracking systems and aplanation tonometry. Results : Patients with Fabry disease had a significant twofold increase in radial artery IMT and distensibility, independent of body surface area, age and mean blood pressure. In both groups, older age at the time of examination was significantly associated with larger radial artery IMT. The relationship between age and radial IMT was 2.3-fold higher in patients with Fabry disease than in controls ( p > 0.01). Carotid IMT was mildly but significantly increased in patients with Fabry disease (+18%), whereas distensibility was unchanged.
Conclusion : This study presents evidence of a major increase in arterial wall thickness and distensibility, measurable at the site of a medium-sized artery, in a cohort of patients with classic Fabry disease.     

Anterior abdominal wall closure in bladder exstrophy

Anterior abdominal wall closure without pelvic osteotomy for bladder exstrophy can be very diffecult and more often than not calls for various reconstructive measures. A technique is described that involves bilateral detachment of the rectus abdominis from the pubic ramus, midline vertical closure, and refixation to the pubic rami after medial and caudal advancement. A relaxing fascial incision may be required. This technique has been used in 15 patients and has obviated the need for pelvic osteotomy, thereby decreasing the postoperative morbidity. It has resulted in good cosmetic repair in all patients in addition to the ease of abdominal wall closure. The healing has been remarkably good. This technique is particularly useful in children coming for primary surgery after the neonatal period. Correspondence to: V. Bhatnagar     

Assessment of thyroid function in pesticide formulators

Thirty male pesticide formulators exposed to the dust and liquid formulation of endosulfan, quinalphos, chlorpyriphos, monocrotophos, lindane, parathion, phorate, and fenvalerate and 20 comparable control subjects from the same area of study were examined for the evaluation of thyroid function tests. The level of TSH was elevated (about 28%) in pesticide formulators as compared to a control group, but the increase was statistically insignificant. Based on the individual TSH measurement, 3 of 30 formulators had isolated elevated levels of TSH and seem to have acquired sub-clinical hypothyroidism; five had TSH values slightly elevated to the upper boarder line (4.03 muIU/ml); and the majority of formulators (N= 22) had TSH values in the normal range varying from 1.29 to 3.9 muIU/ml. Total T3 was suppressed significantly (P< 0.01) in formulators, while marginal decrease (about 7%) was noticed in T4 level. This study indicated thyroid function impairment in few pesticide formulators.     

Long-term prognosis of renal function in boys treated for posterior urethral valves.

This paper discusses the long-term prognosis of renal function in 84 boys treated for posterior urethral valves and followed up for a period ranging from 1-21 years. Thirty-one of the 84 patients (39.3%) were either adolescents or had crossed adolescence and this study highlights the changes through adolescence and puberty. Serum creatinine was found to be raised in 53% patients at presentation and 22.5% patients eventually progressed to chronic renal failure. Serum creatinine value 1 year after appropriate decompression of the urinary tract was a more accurate predictor of the eventual renal outcome rather than serum creatinine at presentation. Decompensation at puberty was detected in 3 patients in this study. The predisposing factors identified were the persistence of gross hydroureteronephrosis with voiding dysfunction after treatment in one patient and renal parenchymal disease in the other 2 patients. The "risk factors" for predicting a poor eventual renal function were persistently raised serum creatinine 1 year after decompression by diversion or fulguration, bilateral high grade vesicoureteral reflux, persistent severe upper tract dilatation after treatment, voiding dysfunction and delayed presentation. This study emphasizes the need to diagnose and intervene early, to investigate post-treatment persistent upper tract dilatation for vesicoureteral junction obstruction and for detrusor dysfunction by a complete urodynamic evaluation and to follow up these patients carefully through adolescence and adulthood.