Increased risk of colorectal cancer associated with congenital anomalies of the urinary tract.

Two young mothers of children presenting with congenital anomalies of the urinary tract underwent colectomy for carcinoma or adenomatosis of the colon. In another family with urinary anomalies, the maternal grandmother had died from carcinoma of the colon at 36 years of age. This previously unreported associated led to two reviews. In the first, of 14 patients with colorectal cancer presenting under 45 years of age, three had known congenital urinary tract anomalies. In the second, which included the first three families, a detailed family history was obtained from children receiving treatment for congenital anomalies of the urinary tract. Seven of 116 grandparents and two mothers among 58 parents had colorectal carcinoma or adenomatosis of the colon; this was significantly greater than the expected incidence. A family history of congenital anomalies of the urinary tract may be a useful marker in screening for colorectal cancer.

Vitamin K coagulation status in surgical newborns and the risk of bleeding.

The vitamin K coagulation status in surgical newborns, who may be at increased risk of developing hypocoagulability and hemorrhage, has not previously been studied. Therefore, we measured the combined activity of the plasma vitamin K-dependent coagulation factors (Thrombotest), total prothrombin, PIVKA II, plasma vitamin K1, fibrinogen, D-Dimer, and platelets in 49 newborns admitted to a neonatal surgical intensive care unit. All infants had significant pathology, and treatment involved surgery in all but two. Twenty-three infants (47%) underwent surgery on two or more occasions. Intravenous or oral antibiotics were used in all patients and many received more than one course. All infants had vitamin K1 prophylaxis at birth. At day 0 (date of birth), the mean Thrombotest and total prothrombin levels were 51% (range, 20% to 100%) and 40% (range, 24% to 59%), respectively. Coagulation activity decreased on day 1 (P > .1) and was followed by a graduate increase in clotting activity, reaching normal adult levels (> 60%) at day 5 for Thrombotest and day 24 for total prothrombin. Only three infants had a Thrombotest less than 20%. PIVKA II was detected in 20 cases (41%). However, levels were within normal limits (< 0.9%) in 17 of these, and between 1.0 and 4.8% in the remaining three infants. There was no relationship between elevated PIVKA levels and coagulation activity in these patients. Plasma vitamin K1 was very high, particularly in the first days of life.(ABSTRACT TRUNCATED AT 250 WORDS)

Total nephrectomy in children: a clinicopathological review.

The records of 85 children undergoing total nephrectomy have been reviewed. Diagnoses ranged between pyelonephritis (37 patients), renal tumours (25), renal dysplasia and multicystic kidney (16), obstructive uropathy (6) and renal vein thrombosis (1). In the pyelonephritic group a contributory ipsilateral abnormality was demonstrated in 36 of the 37 children. Vesicoureteric reflux (VUR) was the most common abnormality in this cohort and was usually of a severe grade. Calculus disease was present in 9 patients, only 1 of whom had VUR. Of the remaining 7 patients with pyelonephritis, 6 had evidence of obstruction to the upper tracts. Calculus disease poses a more severe threat to the childhood kidney than the well known risk factors of vesicoureteric reflux and obstructive uropathy.

Fetal vesicoureteral reflux: outcome following conservative postnatal management.

Of 222 infants with a urinary tract abnormality detected antenatally 30 male and 9 female patients (64 renal units) were found to have primary vesicoureteral reflux. Grade of reflux was predominantly severe, with grade III or higher noted in 83% of the patients. Prenatal and postnatal ultrasound failed to detect any abnormality in 29 refluxing units (45%) discovered contralateral to the known abnormal system, although 19 had grade III or higher reflux. Of the 64 refluxing units 8 underwent primary ureteral reimplantation, 12 were lost to followup and 44 were managed conservatively for a mean of 3.3 years. Reflux ceased in 61% of the cases, improved in 14% and remained unchanged in 23%. In only 1 unit did the grade of reflux increase. Documented urinary tract infection occurred in 6 of the 39 reflux patients. Dimercaptosuccinic acid renography performed in 21 infection-free patients demonstrated global reduction in renal parenchyma in 4 units, focal parenchymal defects in 3 and normal function in 14. Conservative postnatal management of fetal vesicoureteral reflux is justified. Global and focal parenchymal changes can occur in the kidneys of infants with reflux despite the absence of urinary tract infection.

Primary vesicoureteric reflux: treatment with subureteric injection of Polytef paste.

A series of 34 children with 40 primary refluxing ureters were treated endoscopically with a subureteric injection of polytetrafluoroethylene paste (Polytef). The amount injected ranged between 0.1 and 0.8 ml (mean 0.3). A single injection cured the reflux in 26 ureters (65%) and the grade of reflux improved in a further 9 ureters. Seven ureters required a second injection and reflux was cured in 6 of these. The overall cure rate was therefore 80% after the second injection. There was one complication due to self-limiting ureteric obstruction following injection. The procedure is quick, easy to perform and effective. We have some reservations about the long-term efficiency and safety of subureteric Polytef injection in children.

Outcome of antenatally diagnosed pelviureteric junction hydronephrosis.

Over a 6-year period, in a series of 107 consecutive patients with antenatally detected urological anomalies, 45 had pelviureteric junction (PUJ) hydronephrosis (66 units). Of these, 24 units (36%) had obstructive (group I) and 42 (64%) non-obstructive hydronephrosis (group II). Significantly compromised renal function was found only in group I. Treatment in group I was by an early pyeloplasty; in group II the patients were kept under close review. During the 6-year period, 2 units (5%) in group II, deteriorated, 13 (31%) improved and 27 (64%) were unchanged. These results suggest that early in life, antenatally diagnosed PUJ hydronephrosis without obstruction may be a relatively benign condition but will require long-term follow-up in order to determine the natural history of the condition.

Surgical presentation of Kawasaki disease (mucocutaneous lymph node syndrome).

Five patients with Kawasaki disease (mucocutaneous lymph node syndrome) are reported whose varied presentations included acute abdominal pain, peripheral arterial aneurysms, digital gangrene and sterile pyuria and whose presenting pathology ranged from hydrops of the gallbladder to enteric pseudo-obstruction. As the complications of the disease can usually be managed without resort to surgery, which is associated with a mortality rate of up to 25 per cent, the recognition of Kawasaki disease will prevent hazardous and unnecessary laparotomy.

Feeding regimens after pyloromyotomy.

In a prospective randomized study three different feeding regimens after operation were compared in 74 babies with infantile hypertrophic pyloric stenosis: gradual regarding of feeds over 48 h (regimen 1), rapid regarding of feeds over 16 h (regimen 2), and initial starvation followed by full normal feeds at 24 h (regimen 3). No significant difference between the treatment groups was found either in episodes of vomiting after operation (regimen 1, 2.9 episodes in 21 patients; regimen 2, 3.6 episodes in 28 patients; regimen 3, 3.6 episodes in 25 patients) or in the mean duration of postoperative hospital stay (regimen 1, 59.3 h; regimen 2, 47.8 h; regimen 3, 56.7 h). We conclude that vomiting following pyloromyotomy is self-limiting and independent of the timetable or composition of the postoperative dietary regimen.

Fetal vesicoureteric reflux.

We present an analysis of 30 patients with fetal vesicoureteric reflux (VUR) from a series of 107 patients with prenatally diagnosed urinary tract anomalies. In 13 patients (Group 1) the fetal VUR was the only urinary tract abnormality. In 17 patients (Group 2) the fetal VUR was combined with other urinary tract problems. In 14 of 46 refluxing renal units there was no evidence of upper tract dilatation on a pre- and post-natal ultrasound examination. Any infant with postnatal urinary tract dilatation needs full urological investigations, including a micturating cystogram. A normal postnatal ultrasound examination does not exclude fetal VUR.

Reflux nephropathy secondary to intrauterine vesicoureteric reflux.

In 107 infants with 182 antenatally diagnosed urinary tract anomalies, 24 had either unilateral (12) or bilateral (12) vesicoureteric reflux (VUR). The VUR was more common in boys (male to female ratio, 16:8) and usually severe (grades IV [16], III [10], II [4], and I [6]). Intravenous pyelography showed the changes of atrophic pyelonephritis in 10 refluxing units, and in another two with an associated pelviureteric junctional hydronephrosis. Lateral ectopia of the ureteric orifices was noted in six of these 10 refluxing renal units. Isotopic renography showed a reduction in function in nine of the 14 patients examined, ranging between 9% and 41%. (45% and above was considered within the normal range). Only two patients developed a urinary infection before intravenous pyelography or isotopic renography was performed, suggesting that renal changes noted were primary rather than secondary. Findings support the hypothesis that foetal VUR may be a contributing factor in the causation of atrophic pyelonephritis (foetal reflux nephropathy) observed in these patients.

Testicular enlargement as a presenting feature of monocytic leukemia in an infant.

Acute monocytic leukemia is uncommon and unusual in its propensity to present with extramedullary involvement. We describe testicular enlargement in an infant as the presenting feature of this disease.

Vesicoureteric reflux and urinary calculi in children.

We present 5 patients with urinary calculi and vesicoureteric reflux (VUR). In 4 patients the VUR was probably primary and in 1 it was secondary to the calculus disease. The incidence of calculi with reflux is approximately 0.5% whereas the incidence of reflux with calculi is approximately 8%.

Could the stomach site help predict outcome in babies with left sided congenital diaphragmatic hernia diagnosed antenatally?

The site of the stomach in 36 babies presenting postnatally with left sided congenital diaphragmatic hernia (CDH) was assessed as a predictor of outcome. Babies with a thoracic stomach had a higher mortality (P less than .0005), and more frequently developed significant persistent foetal circulation (PFC) (P less than .001), than babies in whom the stomach was normally sited. Normal stomach site was associated with 100% survival and only a 20% incidence of significant PFC. It is possible that stomach site may be the most accurate predictor of outcome in left-sided CDH diagnosed antenatally, and may thus help in planning perinatal and postnatal management. It may also open the door for prenatal surgical correction of CDH by predicting a poor prognostic group or, perhaps more importantly, by predicting those babies with a good prognosis in whom antenatal surgery should not be attempted.