Epidermolysis bullosa dystrophica with urinary tract involvement.

A case of epidermolysis bullosa dystrophica ( EBD ), neonatally corrected atresia of the pylorus and early infantile development of urinary tract manifestations is reported. Owing to misinterpretation of radiological findings, instrumental treatment of the urethra was employed. It resulted in complete and permanent obliteration of the lumen. While the skin disorder has remained slight to moderate in severity, recurrent keratitis has resulted in impaired vision.

Development of hypertension and uraemia after pyelonephritis in childhood: 27 year follow up.

OBJECTIVE: Determination of the long term incidence of uraemia, hypertension, and toxaemia in pregnancy associated with non-obstructive focal renal scarring after pyelonephritis in childhood 25-35 years earlier. DESIGN: 27 Year follow up of patients with non-obstructive focal scarring identified from a retrospective review of intravenous urograms performed in childhood between 1951 and 1967. SETTING: Paediatric primary referral centre and urological clinic in tertiary referral centre. PATIENTS: 30 Patients (mean age 33 (range 22-41] with non-obstructive focal renal scarring first detected between 1951 and 1967 and a history of febrile urinary tract infection. MAIN OUTCOME MEASURE: Hypertension and complications of renal damage. RESULTS: Three patients had developed end stage renal disease, seven had developed hypertension, two of 16 women had a history of toxaemia during pregnancy, and seven patients had undergone renal surgery during follow up. Of the 20 patients who had neither had renal surgery nor had end stage renal disease, all had a significantly lower glomerular filtration rate and renal plasma flow and higher diastolic blood pressure, mean arterial blood pressure, plasma renin activity, and serum beta 2 microglobulin concentration than 13 healthy age matched controls. Diastolic blood pressure and plasma renin activity were positively correlated (r = 0.50, p less than 0.05) and so were fractional sodium excretion and both systolic and diastolic blood pressures (r = 0.54, p less than 0.01, r = 0.51, p less than 0.01 respectively). The progress of renal damage was unrelated to the incidence of recurrent infections. CONCLUSIONS: Children with focal renal scarring due to pyelonephritis are at high risk of serious long term consequences. It is essential that they are given adequate attention and care during adolescence and pregnancy.

[Erroneous diagnosis of nephroblastoma (Wilm's tumor) based on SIOP data]. / Oshibochnyi diagnoz nefroblastomy (opukholi Vil'msa) po materialam SIOP.

Available films of 21 cases of erroneously diagnosed Wilms' tumor in the European Wilms' material have been studied. The diagnosis has been reassessed and the reasons for agreement in 6 cases and disagreement in the remaining 15 are discussed. General diagnostic recommendations are given to help secure optimal diagnostic information.

Micturition cysto-urethrography. Potential diagnostic pitfalls in the male infant.

Some technical aspects of micturition cysto-urethrography, particularly important in the male infant, are emphasized. The catheter used for installation of the contrast medium must be removed before the examination. Unless films are exposed in the true lateral projection and the patient voids with a constant stream, abnormalities of the urethra may easily become overlooked.

Slip of the capital femoral epiphysis: revival of a method of assessment.

A simple and exact method for evaluation of suspected or overt slip of the femoral capital epiphysis is revived. This procedure gives an estimate of all degrees of abnormality from the early stages to those with gross deformity. In our experience slip of the femoral capital epiphysis is always posterior and in approximately 80% of cases bilateral. Therefore, both hips should be examined whenever this condition is suspected. The method provides for examination of each hip separately. Strict adherence to the procedure also provides a precise way of assessing the femoral anteversion angle, significant in many orthopedic disorders. For the latter purpose access to a particular nomogram is mandatory.

Scoliosis in myelomeningocele.

Prevalence, type, and magnitude of scoliosis were studied in 163 patients with myelomeningocele. A scoliosis was diagnosed in 143 of them, congenital in 15 percent of the cases and developmental in the remaining patients. The severity of scoliosis increased with age and was more severe the higher the level of the neurologic deficit. The direction of the curves was correlated with pelvic obliquity, but not with hip dislocation. Although some patients with low level anomalies may develop severe scoliosis, patients with levels above L3 run a considerably higher risk in this respect. The radiographic baseline examination of the spine carried out in the newborn seems to permit a reasonable prognosis in regard to future scoliosis. Gross syringohydromyelia caused or contributed to scoliosis in 5 percent of the cases. However, less extensive syringohydromyelia and Chiari malformations due to abnormal neuromuscular control also promote the development of scoliosis in these patients.

The variability of urographic findings in posterior urethral valves.

The full variety of secondary and associated upper tract abnormalities concurrent with obstructive valves seems not to be generally appreciated. While upper urinary dilatation and impaired renal function are features regarded as almost obligatory, there are cases at the other end of the spectrum with surprisingly slight or no abnormalities of the bladder, ureters or collecting systems. Therefore a negative IVP does not exclude posterior urethral valves in any boy with symptoms pertaining to the urinary tract. A MCU should invariably be included in the radiological work-up.

Conventional abdominal radiography as a means to rule out ileo-caecal intussusception.

The experiences gained with conventional radiographic examinations as a means of excluding childhood ileo-caecal intussusception are presented. According to these, a supplementary barium enema is indicated a) in patients under 6 months of age, b) in patients presenting with scant intestinal gas, c) in patients where the caecum cannot be outlined, d) in patients with a possible mass lesion of the colon, e) in patients with obstruction presumably due to intussusception, and f) in patients with discrepancy between the clinical and radiologic findings (1, 4). In all other patients the barium examination is not used. The key to a successful application of this protocol is a thorough familiarity with the limitations and potentials of the conventional abdominal examination.

Thoracic neuroblastoma of the neonate.

Five cases of neonatal thoracic neuroblastoma were either clinically asymptomatic or presented as uncharacteristic respiratory distress. Chest radiography demonstrated a spectrum of abnormalities varying from a lobulated, apical thoracic mass with or without evidence of expansion, to a spindle-shaped paraspinal soft tissue widening, or a homogeneously dense hemithorax. In the newborn a neoplasm in the posterior mediastinum most likely represents a neuroblastoma, either primary or metastatic. In a high percentage of cases the primary tumour as well as possible skeletal metastases have an increased uptake of radiopharmaceuticals. Therefore a bone scan should be the next step for evaluation of the neoplasm. Supplementary sonographic screening of the retroperitoneum and lesser pelvis is easily performed and may contribute significant information. Urography is hardly mandatory any longer. Metrizamide CT myelography, highly recommended in infants and children, may be omitted in the neonate with no neurologic deficit. Urinary excretion of catecholamine metabolites is usually elevated and must always be determined.

Kidney size and growth in unilateral renal agenesis and in the remaining kidney following nephrectomy for Wilms' tumor.

The kidney size and renal growth were determined in unilateral renal agenesis and in the remaining kidney following nephrectomy for Wilms' tumor. The ultimate length of 98 per cent of the functioning kidneys in renal agenesis is expected not to exceed +5.6 SD. In the tumor series the corresponding figure is +4.2 SD.