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Urologic complications in Menkes' kinky hair disease
90
INTERNATIONAL ABSTRACTS
Renal function remained stable in 20 patients, deteriorated in two, and improved in one. Fourteen patients are dry day and night (eight on clean intermittent catheterization and two with an artificial urinary sphincter), seven are dry by day and wet at night, and two are wet day and night. Of the 23 patients, 15 experienced a postoperative complication, the most common of which was loss of the antireflux ileocecal mechanism in eight. Bladder replacement with an isolated bowel segment is feasible in properly selected patients. This series illustrates many of the areas for consideration in total and subtotal bladder replacement. George Holcomb, Jr
reported 3 to 6 months postnephrectomy in kidney donors. Age at the time of nephrectomy, duration of follow-up, or sex had no influence on the residual creatine clearance. None of these patients had clinically significant hypertension or proteinuria. Since so little evidence of kidney damage could be documented after such a long observation period, hypertension would seem to be seldomly of clinical importance in man unless other factors were present.--Prem Puri
The Upper Renal Tracts in Adolescents and Young Adults with Myelomeningocele. A.M.K. Rickwood and D.G. Thomas. Z Kind-
N Engl J Med 312:1111-1119, (April 12), 1985.
erchir 39 Suppl I I: 104-106, (December), 1984. Thc state of the upper urinary tracts was assessed in 181 adolescents and young adults with myelomeningocele. At the age of 16 years, abnormalities were present in 75 patients (41%). The risk of these complications was related to the extent of the neurological deficit and the presence of a urinary diversion. During follow-up into early adult life, fresh or further deterioriation of the upper renal tracts occurred in 48 patients (27%). The pattern of this deterioriation was influenced by whether or not previous urinary diversion had been performed. Thomas A. Angerpoinmer Percutaneous Nephrostomy in Infants and Children. P. Stanley, J. W. Bear, and B.S. Reid. Am J Roentgenol 141:473-477, (September), 1983.
The authors report their experience with percutaneous nephrostomy drainage in 28 patients ranging in age from 1 day to 18 years. Indications for the procedure included posturologic surgery complications (14), UPJ obstruction (9), tumor obstruction (3), renal dysplasia with ureteric atresia (1), and bladder rupture with posterior urethral valves (1). Infection was present in nine patients, renal failure in six patients, and both complications in four patients. All patients improved following catheter placement. The only major complication (bleeding and sepsis) occurred during a catheter exchange in one patient. The authors describe their technique in detail and illustrate four patients with radiographs and ultrasonograms.--Randall W. Powell Neonatal Hypertension From a Unilateral Multicystic, Dysplastic Kidney. Y. Chen, F.B. Stapleton, S. Roy, I11. et al. J Urol 133:664
665, (April), 1985. Multicystic renal dysplasia is an extremely uncommon cause of hypertension in children, and the few reported cases have not been of newborns. A neonate is described in whom severe hypertension associated with elevated peripheral plasma renin resulted from a unilateral multicystic, dysplastic kidney. Hypertension and plasma renin activity normalized after unilateral nephrectomy. No evidence of perfusion or excretory renal function in the dysplastic kidney was present on a radionuclide renal scan. This child demonstrates a relationship between hypertension and unilateral multicystic renal dysplasia. George Holcomb, Jr Long-term Follow-up of Patients Who Underwent Unilateral Nephrectomy in Childhood. P. Robitail/e, J.G. Mongeau, and L.
Lartie. Lancet 1:1297-1299, (June), 1985. The long-term damaging potential of remnant nephron hyperperfusion was investigated in patients who had undergone unilateral nephrectomy in childhood. Twenty-seven patients were examined after a mean of 23.3 years postnephrectomy. The average creatine clearance was 83.9 - 16.5 m L / m i n / 1 . 7 3 m z or 74.3% of that in healthy controls with two kidneys; it was a value similar to that
A 13-year-old Boy with Aniridia and Proteinuria, 11 Years After N e p h r e c t o m y for a W i l m s Tumor. J.R. Ingelfinger and R.B. Colvin.
This case presentation is that of a 13-year-old boy admitted because of hypertension and proteinuria. In early infancy the child was found to have bilateral aniridia, glaucoma, and cataracts. At age 17 months a right abdominal mass was found and a stage one Wilms' tumor diagnosed. He was treated by right nephrectomy and postoperative Actinomycin D. During the follow-up period the patient had a normal blood pressure, normal urinalysis, and an IVP showing only compensatory hypertrophy of the remaining kidney. At 13 years of age, following a severe deramtitis, the patient was found to have a blood pressure of 170/110 and 4 + protein in his urine. The BUN was 13, Creatinine was 0.6. IVP and ultrasound revealed only compensatory enlargement of the left kidney. A renal biopsy revealed focal segmental glomerulosclerosis. It was the authors' impression that the changes in the "normal" kidney were due to hyperfiltration over the 12 years following nephrectomy. After loss of functioning renal mass, residual renal tissue rapidly undergoes hypertrophy and hyperplasia. In animals in which this has been studied, pathologic alterations gradually appear in the initially normal residual glomeruli eventuating in glomerulosclerosis. This interesting report suggests that unilateral nephrectomy may have eventual deliterous effects on the contralateral kidney.--Eugene S. Wiener Urologic Complications in Menkes" Kinky Hair Disease. T. Oshio,
C. Matsumura, A. Kirino, et al. J J ap Soc Pediatr Surg 21:508 514, 1985. Two cases of Menkes' Kinky Hair Disease with urologic complications are reported. Case 1 had multiple bladder diverticula, right vesicoureteral reflux, and right hydronephrosis. Case 2 had a left nephrectomy at 26 days of age because of massive bleeding. Unlike renal bleeding, bladder diverticula are a known complication in children with Menkes' Kinky Hair Disease. Early diagnosis and management are r e q u i r e d . ~ . Suita MUSCULOSKELETAL SYSTEM Talectomy for Equinovarus Deformity in Myelodysplasia. T. Trum-
he/e, J.B. Banta, J.F. Roycroft, et al. J Bone Joint Surg 67A:16-20, (January), 1985. Tatectomy was performed in nine patients (17 feet), for myelomeningocele equinovarus deformity. Follow-up was 7 years. Complications included four residual talar fragments, one persistent forefoot adduction, one pin tract infection, and one proximal migration of a fixation pin in a noncompliant patient. Six feet were left incompletely corrected because of residual forefoot deformity. The authors conclude that the procedure is worthwhile doing if there is careful attention to detail.--Anthony H. Alter Split Posterior Tibial Tendon Transfers in Children with Cerebral Spastic Paralysis and Equinovarus Deformity. T.F. Kling, H. Kauf-
er, and R.M. Hensinger. J Bone Joint Surg 67A:186-194, (February), 1985.
INTERNATIONAL ABSTRACTS
Renal function remained stable in 20 patients, deteriorated in two, and improved in one. Fourteen patients are dry day and night (eight on clean intermittent catheterization and two with an artificial urinary sphincter), seven are dry by day and wet at night, and two are wet day and night. Of the 23 patients, 15 experienced a postoperative complication, the most common of which was loss of the antireflux ileocecal mechanism in eight. Bladder replacement with an isolated bowel segment is feasible in properly selected patients. This series illustrates many of the areas for consideration in total and subtotal bladder replacement. George Holcomb, Jr
reported 3 to 6 months postnephrectomy in kidney donors. Age at the time of nephrectomy, duration of follow-up, or sex had no influence on the residual creatine clearance. None of these patients had clinically significant hypertension or proteinuria. Since so little evidence of kidney damage could be documented after such a long observation period, hypertension would seem to be seldomly of clinical importance in man unless other factors were present.--Prem Puri
The Upper Renal Tracts in Adolescents and Young Adults with Myelomeningocele. A.M.K. Rickwood and D.G. Thomas. Z Kind-
N Engl J Med 312:1111-1119, (April 12), 1985.
erchir 39 Suppl I I: 104-106, (December), 1984. Thc state of the upper urinary tracts was assessed in 181 adolescents and young adults with myelomeningocele. At the age of 16 years, abnormalities were present in 75 patients (41%). The risk of these complications was related to the extent of the neurological deficit and the presence of a urinary diversion. During follow-up into early adult life, fresh or further deterioriation of the upper renal tracts occurred in 48 patients (27%). The pattern of this deterioriation was influenced by whether or not previous urinary diversion had been performed. Thomas A. Angerpoinmer Percutaneous Nephrostomy in Infants and Children. P. Stanley, J. W. Bear, and B.S. Reid. Am J Roentgenol 141:473-477, (September), 1983.
The authors report their experience with percutaneous nephrostomy drainage in 28 patients ranging in age from 1 day to 18 years. Indications for the procedure included posturologic surgery complications (14), UPJ obstruction (9), tumor obstruction (3), renal dysplasia with ureteric atresia (1), and bladder rupture with posterior urethral valves (1). Infection was present in nine patients, renal failure in six patients, and both complications in four patients. All patients improved following catheter placement. The only major complication (bleeding and sepsis) occurred during a catheter exchange in one patient. The authors describe their technique in detail and illustrate four patients with radiographs and ultrasonograms.--Randall W. Powell Neonatal Hypertension From a Unilateral Multicystic, Dysplastic Kidney. Y. Chen, F.B. Stapleton, S. Roy, I11. et al. J Urol 133:664
665, (April), 1985. Multicystic renal dysplasia is an extremely uncommon cause of hypertension in children, and the few reported cases have not been of newborns. A neonate is described in whom severe hypertension associated with elevated peripheral plasma renin resulted from a unilateral multicystic, dysplastic kidney. Hypertension and plasma renin activity normalized after unilateral nephrectomy. No evidence of perfusion or excretory renal function in the dysplastic kidney was present on a radionuclide renal scan. This child demonstrates a relationship between hypertension and unilateral multicystic renal dysplasia. George Holcomb, Jr Long-term Follow-up of Patients Who Underwent Unilateral Nephrectomy in Childhood. P. Robitail/e, J.G. Mongeau, and L.
Lartie. Lancet 1:1297-1299, (June), 1985. The long-term damaging potential of remnant nephron hyperperfusion was investigated in patients who had undergone unilateral nephrectomy in childhood. Twenty-seven patients were examined after a mean of 23.3 years postnephrectomy. The average creatine clearance was 83.9 - 16.5 m L / m i n / 1 . 7 3 m z or 74.3% of that in healthy controls with two kidneys; it was a value similar to that
A 13-year-old Boy with Aniridia and Proteinuria, 11 Years After N e p h r e c t o m y for a W i l m s Tumor. J.R. Ingelfinger and R.B. Colvin.
This case presentation is that of a 13-year-old boy admitted because of hypertension and proteinuria. In early infancy the child was found to have bilateral aniridia, glaucoma, and cataracts. At age 17 months a right abdominal mass was found and a stage one Wilms' tumor diagnosed. He was treated by right nephrectomy and postoperative Actinomycin D. During the follow-up period the patient had a normal blood pressure, normal urinalysis, and an IVP showing only compensatory hypertrophy of the remaining kidney. At 13 years of age, following a severe deramtitis, the patient was found to have a blood pressure of 170/110 and 4 + protein in his urine. The BUN was 13, Creatinine was 0.6. IVP and ultrasound revealed only compensatory enlargement of the left kidney. A renal biopsy revealed focal segmental glomerulosclerosis. It was the authors' impression that the changes in the "normal" kidney were due to hyperfiltration over the 12 years following nephrectomy. After loss of functioning renal mass, residual renal tissue rapidly undergoes hypertrophy and hyperplasia. In animals in which this has been studied, pathologic alterations gradually appear in the initially normal residual glomeruli eventuating in glomerulosclerosis. This interesting report suggests that unilateral nephrectomy may have eventual deliterous effects on the contralateral kidney.--Eugene S. Wiener Urologic Complications in Menkes" Kinky Hair Disease. T. Oshio,
C. Matsumura, A. Kirino, et al. J J ap Soc Pediatr Surg 21:508 514, 1985. Two cases of Menkes' Kinky Hair Disease with urologic complications are reported. Case 1 had multiple bladder diverticula, right vesicoureteral reflux, and right hydronephrosis. Case 2 had a left nephrectomy at 26 days of age because of massive bleeding. Unlike renal bleeding, bladder diverticula are a known complication in children with Menkes' Kinky Hair Disease. Early diagnosis and management are r e q u i r e d . ~ . Suita MUSCULOSKELETAL SYSTEM Talectomy for Equinovarus Deformity in Myelodysplasia. T. Trum-
he/e, J.B. Banta, J.F. Roycroft, et al. J Bone Joint Surg 67A:16-20, (January), 1985. Tatectomy was performed in nine patients (17 feet), for myelomeningocele equinovarus deformity. Follow-up was 7 years. Complications included four residual talar fragments, one persistent forefoot adduction, one pin tract infection, and one proximal migration of a fixation pin in a noncompliant patient. Six feet were left incompletely corrected because of residual forefoot deformity. The authors conclude that the procedure is worthwhile doing if there is careful attention to detail.--Anthony H. Alter Split Posterior Tibial Tendon Transfers in Children with Cerebral Spastic Paralysis and Equinovarus Deformity. T.F. Kling, H. Kauf-
er, and R.M. Hensinger. J Bone Joint Surg 67A:186-194, (February), 1985.