- Email: [email protected]
A Broader Spectrum of Abnormalities in the Prune Belly Syndrome
0022-534 7/86/1352-0324$02.00 /0 Vol. 135, February Printed in U.S.A.
THE JOURNAL OF UROLOGY
Copyright© 1986 by The Williams & Wilkins Co.
A BROADER SPECTRUM OF ABNORMALITIES IN THE PRUNE BELLY SYNDROME DENIS F. GEARY, IAN B. MAcLUSKY, BERNARD M. CHURCHILL AND GORDON McLORIE From the Nephrology and Respiratory Divisions, Department of Pediatrics, and Urology Division, Department of Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada
ABSTRACT
The clinical course of 25 children with the prune belly syndrome was reviewed retrospectively to assess the over-all morbidity associated with this disorder. There were 3 neonatal deaths of renal or pulmonary disease. Chronic renal insufficiency or end stage renal disease developed in 5 survivors, all of whom had impaired kidney function in early infancy. An additional 17 patients survived with only mild renal insufficiency. Growth retardation, which correlated poorly with renal function, was present in a third of the patients. Clinically significant pulmonary and orthopedic problems were noted in 55 per cent of the survivors. Chronic constipation was another common, although less serious, feature. This report emphasizes the severity of the extrarenal problems associated with the prune belly syndrome. The prune belly syndrome usually is described as a deficiency of the anterior abdominal muscle, with complex genitourinary malformations and bilateral undescended testes. '- 3 Such a description allows recognition of all such patients and lays appropriate emphasis on the urinary tract abnormalities from which most of the morbidity and mortality associated with the syndrome ensue. Accordingly, reports in the literature have concentrated primarily on urological disorders and provide little information on the several other features of the syndrome. When the extrarenal factors have been described, 1 • 2• 4 • 5 there has been little emphasis on their contribution to the over-all morbidity associated with the syndrome. We describe the clinical course of 25 patients seen between 1970 and 1982, and stress those features that do not involve the genitourinary tract. PATIENTS AND METHODS
The charts of all patients with the prune belly syndrome seen at this hospital between 1970 and 1982 were reviewed. All 25 such patients identified had absent abdominal muscles, typical urinary tract abnormalities and bilateral cryptorchidism. We reviewed the clinical outcome in the patients who survived, with particular attention paid to growth, respiratory, orthopedic, gastrointestinal and developmental problems. Initial renal function was estimated from plasma creatinine values obtained when the patients were clinically stable after their first presentation to this hospital. Final renal function was estimated from the most recently recorded plasma creatinine value. Chronic renal insufficiency was arbitrarily defined as a plasma creatinine value greater than 1. 7 mg./dl. (150 µmol./ 1.) and end stage renal disease was defined as the time dialysis was begun. Growth was evaluated from the most recently recorded heights. Since documentation was not available in all cases, we assumed that supine measurements were taken in patients less than 2 years old and standing heights were obtained in older patients. Growth was expressed as height standard deviation scores derived from the table of Tanner and associates. 6 The relationship between growth and renal function was evaluated by a comparison of plasma creatinine with final recorded heights. Respiratory problems were classified as recurrent bronchitis Accepted for publication October 16, 1985. Read at annual meeting of American Urological Association, Atlanta, Georgia, May 12-16, 1985.
324
when repeated episodes of respiratory insufficiency associated with respiratory tract infections were documented, formal pulmonary function testing indicated restrictive or obstructive airway disease, or chronic bronchodilator therapy was required. We described patients as having postoperative respiratory distress if they required treatment (for ,example croup tent or bronchodilators) for more than 24 hours postoperatively or had respiratory depression that persisted after reversal of anesthesia. Neonatal respiratory problems were considered individually. Orthopedic abnormalities and the difficulties encountered in their treatment are described from information recorded by the orthopedic consultants who cared for the patients. Gastrointestinal problems were of 2 types: congenital malformations requiring an operation in the neonatal period and long-standing constipation. Formal developmental testing was done on 5 children and was estimated from the histories or level achieved at school in a further 8. RESULTS
Twenty patients presented to us in the neonatal period, while the remainder were referred when they were 3 months to 15 years old. Two patients were twins whose siblings were unaffected. Mean age at followup for the surviving children was 8.45 years (range 0.52 to 18. 74 years). Mortality. There were 3 neonatal deaths: 1 of renal failure associated with severe renal dysplasia and hypoplastic lungs that were noted at autopsy, 1 of hypoplastic lungs in the presence of normal renal function and 1 of renal failure associated with multiple cardiac anomalies. Renal function. Chronic renal insufficiency or end stage renal disease developed in 5 survivors (23 per cent), including 4 in whom azotemia was present soon after birth. No information was available before adolescence in the fifth patient. Growth. Inadequate growth (height standard deviation scores more than 2 standard deviations below the mean for chronological age) was observed in 7 of the 22 surviving patients (32 per cent) and in 4 of 17 (24 per cent) who did not have chronic renal insufficiency or end stage renal disease. There was a poor correlation between height achieved and final plasma creatinine value (r = -0.39, p >0.05). More detailed information on growth (in 14 patients for whom such information was available) is illustrated in the table-the decrease in height standard deviation scores noted at 1 year, with subsequent improvement,
PRUNE BELLY SYNDROME IN 25 PATIENTS HSDS
5 • CRI/ESRD
4
o CREAT.<1.7 mg./dl
3 2
0
0
0
0
0
0
-2
•
-3
•
0
-1
0
0
0
•
0
0
0 0
0 0
•
0
-4
•
-5
0
0
2
3
4
5
6
7
8
9 10 11 12 13 14 15 16 17
AGE (years) Final height in 22 children with prune belly syndrome. HSDS, height standard deviation scores. CRI, chronic renal insufficiency. ESRD, end stage renal disease. CREAT., creatinine.
Growth in 14 children with the prune belly syndrome Birth Height standard deviation score: Mean A mean
Final
1 Yr.
-1.64
-1.07
-0.57
-1.18
+0.46
indicates that growth velocity was subnormal in the first year of life and that some degree of catch up growth occurred later. Respiratory probkms. One patient died of respiratory failure in the neonatal period, while a second was found at autopsy to have hypoplastic lungs. Recurrent bronchitis developed in 6 patients, while postoperative respiratory distress occurred in 4 children, 3 of whom have no chronic respiratory problems. Pneumothorax developed in 2 neonates and a third patient had pneumonia during the neonatal period. Orthopedic problems. Lower limb abnormalities were observed in 7 patients. Three patients had mild talipes that were corrected by manipulation. One patient had a more severe talipes equinovarus deformity associated with absence of the toes, and eventually required amputation of the foot. Congenital hip dislocation occurred in 4 patients and was associated with dysplasia of the femoral head or acetabulum in 3. Eventually, avascular necrosis of the femoral head developed in 2 patients. Gastrointestinal problems. Intestinal malrotation was noted in 1 of the neonates who died, while another child required a colostomy for meconium ileus obstruction. A further 5 children required continuous treatment for constipation. Development. Developmental delay was documented formally in the 5 children selected for testing and was suspected from the history in another child. No information was recorded regarding school and developmental progress in 9 patients. Details regarding the management or outcome of the genitourinary malformations and testes in these patients are beyond the scope of this report. DISCUSSION
It is clear from our review that the prune belly syndrome involves many organ systems. As reported previously, most morbidity and mortality result from the genitourinary malformations.1-3 However, the presence of clinically significant respiratory or orthopedic abnormalities in 12 of our surviving children (55 per cent) emphasizes the importance of these extrarenal features. In addition, if those patients with growth retardation, developmental delay and gastrointestinal disorders are included then 16 of 22 children (73 per cent) manifest
325
clinically significant nonurological features. Use of the term triad syndrome3 to describe patients with the prune belly syndrome ignores these multisystem abnormalities and should be avoided. Chronic renal insufficiency or end stage renal disease developed in 5 of our surviving patients (23 per cent) during a followup of 8.0 ± 5.5 (mean ± standard deviation) years. This deterioration occurred only in patients whose renal function already was impaired at the initial evaluation. Thus, as suggested previously,1 in the absence of severe renal hypoplasia or dysplasia, nearly normal renal function may be maintained in these children for many years. Normal growth has been reported in patients with the prune belly syndrome despite gross urinary tract abnormalities, unless prevented by extrarenal disorders. 1 However, inadequate growth was seen in 32 per cent of our survivors, 2 of whom had neither chronic renal insufficiency nor any nonurological man ifestations. The poor correlation between growth and plasma creatinine values was of particular interest and would have been more striking if 2 dialysis-dependent patients had been excluded. This finding supports the conclusion of Kleinknect7 and Rizzoni8 and their associates that growth in children with chronic renal failure is unrelated to the severity of the renal functional impairment. Also, the pattern of growth that we observed, with impairment in the first year of life and subsequent stabilization but incomplete catch up growth, has been described previously 7• 8 and emphasizes the need to detect any deterioration in growth during early infancy when appropriate corrective measures still may be beneficial. We did not have enough data to comment on pubertal development in our patients, despite the importance of such information in children with this syndrome. Information about psychomotor development also was incomplete but it is disturbing that 6 children probably were developmentally delayed, while no information was recorded for almost half of the patients. Our use of the term recurrent bronchitis to describe the respiratory disorder found in these patients is imprecise but reflects the variety of nonspecific although clinically significant respiratory problems. Certainly, these children appear unduly susceptible to the development of respiratory insufficiency following upper respiratory tract infections or anesthetics. Several of them had marked thoracic cage deformities (for example kyphoscoliosis, pectus excavatum), and less specific flattening of the thoracic cage with poor rib movement was common. Formal pulmonary function testing was performed in only 2 patients who had low to normal total lung capacity with reduced respiratory flow rates and markedly reduced forced vital capacities. Therefore, we hypothesize that the susceptibility of these patients to respiratory difficulty results from mechanical restriction associated with thoracic cage deformities, which in turn result from the deficiency of abdominal musculature. The occurrence of respiratory depression following anesthesia has been reported previously1 and if not anticipated may result in severe hypoxia with serious medicolegal consequences. It is particularly disturbing that postoperative respiratory distress developed in 3 of our patients with no history of pulmonary problems. The orthopedic abnormalities as reported previously2 • 5 • 9 and noted in our patients principally involve the chest, spine and lower extremities. However, the severity of these defects and the difficulty encountered in their treatment have not been emphasized sufficiently. Extensive treatment was required for correction of dislocated hips in 3 patients. Treatment was impeded by associated renal disease, since abdominal casts were poorly tolerated following urinary diversion procedures. In addition, drainage of the hypoperistaltic upper tracts may be impeded by the application of Gallow's traction. One child eventually required an open reduction and osteotomy when the patient was 16 months old and a vascular necrosis of the femoral
326
GEARY AND ASSOCIATES
head developed in 2 others. The problems encountered in our patients suggest that standard orthopedic principles of treatment may prove inadequate despite previous reports to the contrary. 9 IMPLICATIONS
We document the occurrence of nonurological problems in almost three-quarters of our patients with the prune belly syndrome, and clinicians should be alerted to anticipate such problems. Chronic renal disease remains the major cause of morbidity and mortality in children with this disorder but it is encouraging to note that with appropriate care most patients retain adequate renal function for many years, unless severe renal impairment is noted in infancy. REFERENCES
1. Woodhouse, C.R. J., Ransley, P. G. and Innes-Williams, D.: Prune belly syndrome-report of 47 cases. Arch. Dis. Child., 57: 856, 1982. 2. Rogers, L. W. and Ostrow, P. T.: The prune belly syndrome. Report of 20 cases and description of a lethal variant. J. Ped., 83: 786, 1973.
3. Nunn, I. N. and Stephens, F. D.: The triad syndrome: a composite anomaly of the abdominal wall, urinary system and testes. J. Urol., 86: 782, 1961. 4. Silverman, F. N. and Huang, N.: Congenital absence of the abdominal muscles, associated with malformation of the genitourinary and alimentary tracts; report of cases and review of literature. Amer. J. Dis. Child., 80: 91, 1950. 5. Lattimer, J. K.: Congenital deficiency of the abdominal musculature and associated genitourinary anomalies: a report of 22 cases. J. Urol., 79: 343, 1958. 6. Tanner, J. M., Whitehouse, R. H. and Takaishi, M.: Standards from birth to maturity for height, weight, height velocity, and weight velocity: British children, 1965. IL Arch. Dis. Child., 41: 613, 1966. 7. Kleinknect, C., Broyer, M., Huot, D., Marti-Henneberg, C. and Dartois, A.-M.: Growth and development of non dialyzed children with chronic renal failure. Kidney Int., suppl. 15, 24: S40, 1983. 8. Rizzoni, G., Basso, T. and Setari, M.: Growth in children with chronic renal failure on conservative treatment. Kidney Int., 26: 52, 1984. 9. Tuch, B. A. and Smith, T. K.: Prune belly syndrome: a report of twelve cases and review of the literature. J. Bone Joint Surg., 60: 109, 1978.
THE JOURNAL OF UROLOGY
Copyright© 1986 by The Williams & Wilkins Co.
A BROADER SPECTRUM OF ABNORMALITIES IN THE PRUNE BELLY SYNDROME DENIS F. GEARY, IAN B. MAcLUSKY, BERNARD M. CHURCHILL AND GORDON McLORIE From the Nephrology and Respiratory Divisions, Department of Pediatrics, and Urology Division, Department of Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada
ABSTRACT
The clinical course of 25 children with the prune belly syndrome was reviewed retrospectively to assess the over-all morbidity associated with this disorder. There were 3 neonatal deaths of renal or pulmonary disease. Chronic renal insufficiency or end stage renal disease developed in 5 survivors, all of whom had impaired kidney function in early infancy. An additional 17 patients survived with only mild renal insufficiency. Growth retardation, which correlated poorly with renal function, was present in a third of the patients. Clinically significant pulmonary and orthopedic problems were noted in 55 per cent of the survivors. Chronic constipation was another common, although less serious, feature. This report emphasizes the severity of the extrarenal problems associated with the prune belly syndrome. The prune belly syndrome usually is described as a deficiency of the anterior abdominal muscle, with complex genitourinary malformations and bilateral undescended testes. '- 3 Such a description allows recognition of all such patients and lays appropriate emphasis on the urinary tract abnormalities from which most of the morbidity and mortality associated with the syndrome ensue. Accordingly, reports in the literature have concentrated primarily on urological disorders and provide little information on the several other features of the syndrome. When the extrarenal factors have been described, 1 • 2• 4 • 5 there has been little emphasis on their contribution to the over-all morbidity associated with the syndrome. We describe the clinical course of 25 patients seen between 1970 and 1982, and stress those features that do not involve the genitourinary tract. PATIENTS AND METHODS
The charts of all patients with the prune belly syndrome seen at this hospital between 1970 and 1982 were reviewed. All 25 such patients identified had absent abdominal muscles, typical urinary tract abnormalities and bilateral cryptorchidism. We reviewed the clinical outcome in the patients who survived, with particular attention paid to growth, respiratory, orthopedic, gastrointestinal and developmental problems. Initial renal function was estimated from plasma creatinine values obtained when the patients were clinically stable after their first presentation to this hospital. Final renal function was estimated from the most recently recorded plasma creatinine value. Chronic renal insufficiency was arbitrarily defined as a plasma creatinine value greater than 1. 7 mg./dl. (150 µmol./ 1.) and end stage renal disease was defined as the time dialysis was begun. Growth was evaluated from the most recently recorded heights. Since documentation was not available in all cases, we assumed that supine measurements were taken in patients less than 2 years old and standing heights were obtained in older patients. Growth was expressed as height standard deviation scores derived from the table of Tanner and associates. 6 The relationship between growth and renal function was evaluated by a comparison of plasma creatinine with final recorded heights. Respiratory problems were classified as recurrent bronchitis Accepted for publication October 16, 1985. Read at annual meeting of American Urological Association, Atlanta, Georgia, May 12-16, 1985.
324
when repeated episodes of respiratory insufficiency associated with respiratory tract infections were documented, formal pulmonary function testing indicated restrictive or obstructive airway disease, or chronic bronchodilator therapy was required. We described patients as having postoperative respiratory distress if they required treatment (for ,example croup tent or bronchodilators) for more than 24 hours postoperatively or had respiratory depression that persisted after reversal of anesthesia. Neonatal respiratory problems were considered individually. Orthopedic abnormalities and the difficulties encountered in their treatment are described from information recorded by the orthopedic consultants who cared for the patients. Gastrointestinal problems were of 2 types: congenital malformations requiring an operation in the neonatal period and long-standing constipation. Formal developmental testing was done on 5 children and was estimated from the histories or level achieved at school in a further 8. RESULTS
Twenty patients presented to us in the neonatal period, while the remainder were referred when they were 3 months to 15 years old. Two patients were twins whose siblings were unaffected. Mean age at followup for the surviving children was 8.45 years (range 0.52 to 18. 74 years). Mortality. There were 3 neonatal deaths: 1 of renal failure associated with severe renal dysplasia and hypoplastic lungs that were noted at autopsy, 1 of hypoplastic lungs in the presence of normal renal function and 1 of renal failure associated with multiple cardiac anomalies. Renal function. Chronic renal insufficiency or end stage renal disease developed in 5 survivors (23 per cent), including 4 in whom azotemia was present soon after birth. No information was available before adolescence in the fifth patient. Growth. Inadequate growth (height standard deviation scores more than 2 standard deviations below the mean for chronological age) was observed in 7 of the 22 surviving patients (32 per cent) and in 4 of 17 (24 per cent) who did not have chronic renal insufficiency or end stage renal disease. There was a poor correlation between height achieved and final plasma creatinine value (r = -0.39, p >0.05). More detailed information on growth (in 14 patients for whom such information was available) is illustrated in the table-the decrease in height standard deviation scores noted at 1 year, with subsequent improvement,
PRUNE BELLY SYNDROME IN 25 PATIENTS HSDS
5 • CRI/ESRD
4
o CREAT.<1.7 mg./dl
3 2
0
0
0
0
0
0
-2
•
-3
•
0
-1
0
0
0
•
0
0
0 0
0 0
•
0
-4
•
-5
0
0
2
3
4
5
6
7
8
9 10 11 12 13 14 15 16 17
AGE (years) Final height in 22 children with prune belly syndrome. HSDS, height standard deviation scores. CRI, chronic renal insufficiency. ESRD, end stage renal disease. CREAT., creatinine.
Growth in 14 children with the prune belly syndrome Birth Height standard deviation score: Mean A mean
Final
1 Yr.
-1.64
-1.07
-0.57
-1.18
+0.46
indicates that growth velocity was subnormal in the first year of life and that some degree of catch up growth occurred later. Respiratory probkms. One patient died of respiratory failure in the neonatal period, while a second was found at autopsy to have hypoplastic lungs. Recurrent bronchitis developed in 6 patients, while postoperative respiratory distress occurred in 4 children, 3 of whom have no chronic respiratory problems. Pneumothorax developed in 2 neonates and a third patient had pneumonia during the neonatal period. Orthopedic problems. Lower limb abnormalities were observed in 7 patients. Three patients had mild talipes that were corrected by manipulation. One patient had a more severe talipes equinovarus deformity associated with absence of the toes, and eventually required amputation of the foot. Congenital hip dislocation occurred in 4 patients and was associated with dysplasia of the femoral head or acetabulum in 3. Eventually, avascular necrosis of the femoral head developed in 2 patients. Gastrointestinal problems. Intestinal malrotation was noted in 1 of the neonates who died, while another child required a colostomy for meconium ileus obstruction. A further 5 children required continuous treatment for constipation. Development. Developmental delay was documented formally in the 5 children selected for testing and was suspected from the history in another child. No information was recorded regarding school and developmental progress in 9 patients. Details regarding the management or outcome of the genitourinary malformations and testes in these patients are beyond the scope of this report. DISCUSSION
It is clear from our review that the prune belly syndrome involves many organ systems. As reported previously, most morbidity and mortality result from the genitourinary malformations.1-3 However, the presence of clinically significant respiratory or orthopedic abnormalities in 12 of our surviving children (55 per cent) emphasizes the importance of these extrarenal features. In addition, if those patients with growth retardation, developmental delay and gastrointestinal disorders are included then 16 of 22 children (73 per cent) manifest
325
clinically significant nonurological features. Use of the term triad syndrome3 to describe patients with the prune belly syndrome ignores these multisystem abnormalities and should be avoided. Chronic renal insufficiency or end stage renal disease developed in 5 of our surviving patients (23 per cent) during a followup of 8.0 ± 5.5 (mean ± standard deviation) years. This deterioration occurred only in patients whose renal function already was impaired at the initial evaluation. Thus, as suggested previously,1 in the absence of severe renal hypoplasia or dysplasia, nearly normal renal function may be maintained in these children for many years. Normal growth has been reported in patients with the prune belly syndrome despite gross urinary tract abnormalities, unless prevented by extrarenal disorders. 1 However, inadequate growth was seen in 32 per cent of our survivors, 2 of whom had neither chronic renal insufficiency nor any nonurological man ifestations. The poor correlation between growth and plasma creatinine values was of particular interest and would have been more striking if 2 dialysis-dependent patients had been excluded. This finding supports the conclusion of Kleinknect7 and Rizzoni8 and their associates that growth in children with chronic renal failure is unrelated to the severity of the renal functional impairment. Also, the pattern of growth that we observed, with impairment in the first year of life and subsequent stabilization but incomplete catch up growth, has been described previously 7• 8 and emphasizes the need to detect any deterioration in growth during early infancy when appropriate corrective measures still may be beneficial. We did not have enough data to comment on pubertal development in our patients, despite the importance of such information in children with this syndrome. Information about psychomotor development also was incomplete but it is disturbing that 6 children probably were developmentally delayed, while no information was recorded for almost half of the patients. Our use of the term recurrent bronchitis to describe the respiratory disorder found in these patients is imprecise but reflects the variety of nonspecific although clinically significant respiratory problems. Certainly, these children appear unduly susceptible to the development of respiratory insufficiency following upper respiratory tract infections or anesthetics. Several of them had marked thoracic cage deformities (for example kyphoscoliosis, pectus excavatum), and less specific flattening of the thoracic cage with poor rib movement was common. Formal pulmonary function testing was performed in only 2 patients who had low to normal total lung capacity with reduced respiratory flow rates and markedly reduced forced vital capacities. Therefore, we hypothesize that the susceptibility of these patients to respiratory difficulty results from mechanical restriction associated with thoracic cage deformities, which in turn result from the deficiency of abdominal musculature. The occurrence of respiratory depression following anesthesia has been reported previously1 and if not anticipated may result in severe hypoxia with serious medicolegal consequences. It is particularly disturbing that postoperative respiratory distress developed in 3 of our patients with no history of pulmonary problems. The orthopedic abnormalities as reported previously2 • 5 • 9 and noted in our patients principally involve the chest, spine and lower extremities. However, the severity of these defects and the difficulty encountered in their treatment have not been emphasized sufficiently. Extensive treatment was required for correction of dislocated hips in 3 patients. Treatment was impeded by associated renal disease, since abdominal casts were poorly tolerated following urinary diversion procedures. In addition, drainage of the hypoperistaltic upper tracts may be impeded by the application of Gallow's traction. One child eventually required an open reduction and osteotomy when the patient was 16 months old and a vascular necrosis of the femoral
326
GEARY AND ASSOCIATES
head developed in 2 others. The problems encountered in our patients suggest that standard orthopedic principles of treatment may prove inadequate despite previous reports to the contrary. 9 IMPLICATIONS
We document the occurrence of nonurological problems in almost three-quarters of our patients with the prune belly syndrome, and clinicians should be alerted to anticipate such problems. Chronic renal disease remains the major cause of morbidity and mortality in children with this disorder but it is encouraging to note that with appropriate care most patients retain adequate renal function for many years, unless severe renal impairment is noted in infancy. REFERENCES
1. Woodhouse, C.R. J., Ransley, P. G. and Innes-Williams, D.: Prune belly syndrome-report of 47 cases. Arch. Dis. Child., 57: 856, 1982. 2. Rogers, L. W. and Ostrow, P. T.: The prune belly syndrome. Report of 20 cases and description of a lethal variant. J. Ped., 83: 786, 1973.
3. Nunn, I. N. and Stephens, F. D.: The triad syndrome: a composite anomaly of the abdominal wall, urinary system and testes. J. Urol., 86: 782, 1961. 4. Silverman, F. N. and Huang, N.: Congenital absence of the abdominal muscles, associated with malformation of the genitourinary and alimentary tracts; report of cases and review of literature. Amer. J. Dis. Child., 80: 91, 1950. 5. Lattimer, J. K.: Congenital deficiency of the abdominal musculature and associated genitourinary anomalies: a report of 22 cases. J. Urol., 79: 343, 1958. 6. Tanner, J. M., Whitehouse, R. H. and Takaishi, M.: Standards from birth to maturity for height, weight, height velocity, and weight velocity: British children, 1965. IL Arch. Dis. Child., 41: 613, 1966. 7. Kleinknect, C., Broyer, M., Huot, D., Marti-Henneberg, C. and Dartois, A.-M.: Growth and development of non dialyzed children with chronic renal failure. Kidney Int., suppl. 15, 24: S40, 1983. 8. Rizzoni, G., Basso, T. and Setari, M.: Growth in children with chronic renal failure on conservative treatment. Kidney Int., 26: 52, 1984. 9. Tuch, B. A. and Smith, T. K.: Prune belly syndrome: a report of twelve cases and review of the literature. J. Bone Joint Surg., 60: 109, 1978.