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Hemolytic-Uremic Syndrome in Two Siblings From a Nonendemic Area
402
PEDIATRIC UROLOGY
versity of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania
Cleveland; and Department of Biometry, Case Western Reserve University, School of Medicine, Cleveland, Ohio
Pediatrics,68:45-49 (July) 1981
J. Ped. Surg., suppl. 1, 16: 583-586 (Aug.) 1981
Fourteen children from 5 to 17 years old with meningomyelocele and significant fecal soiling were examined by 3-balloon anorectal manometry. Eight patients who demonstrated rectal sensation were selected for biofeedback conditioning in an effort to improve fecal continence. Since all children were shown to have absent external sphincter responses reflexively and on voluntary command improved continence depended on learning to increase rectal tone by contracting gluteal and related muscles. Of the 8 patients 4 had significant improvement in fecal continence (2 continent, 1 improved 90 per cent and 1 improved 75 per cent). N euromotor testing was not reliable in determining potential candidates for successful biofeedback training but no patient who responded well had a defect proximal to L5. Sensory awareness of rectal distension and ability to contract gluteal or related muscles were essential for this training to be effective. H.M.S. 2 tables, 8 references
The best incision is one that provides optimal exposure with minimal trauma, allows extension, provides greatest tensile strength and maximizes patient comfort and cosmetic end result. The exposure and the ability to extend the incision are related to the diameter or shape of the cavity under consideration. The authors correlated the topography of the abdominal wall with growth. There were 91 children studied. None was markedly obese or cachectic and none had an abdominal wall defect or abdominal distension. In full-term children, when compared to the standard growth curves, the slope of the measurements of the abdominal wall showed a much smaller slope. This was especially true of a measurement of the costoiliac difference when there was practically no growth from birth to 16 years old. In premature infants the same general trend was observed although the curves were different. The authors concluded that the younger the child the relatively larger the abdominal cavity and abdominal wall. Both gridiron and transverse abdominal incisions have been shown to be superior to vertical celiotomy in children and adults. Infants, premature newborns and small children have a disproportionately large abdomen and liver. The transverse diameter of the abdomen is more pronounced in infants so that the abdominal cavity has an ellipsoidal shape. Consequently, the space for extension of vertical incision is somewhat limited, while lateral extension newborns are more easily achieved. The low position of the umbilicus in infants places a transverse supraumbilical incision in the anatomical center of the abdomen. An additional advantage of this incision is that there is virtually no need for selfretaining retractors and less relaxation is needed for operative exposure. The cosmetic advantage of placing an incision in the skin tension lines is well recognized. G. W.K. 4 figures, 23 references
Editorial comment. This seems a useful technique. Manometrics quickly identify the patients who can contract the sphincters somewhat and half of these benefited from training to a quite useful degree. L.R.K. Hemolytic-Uremic Syndrome in Two Siblings From a Nonendemic Area B. L. WARSHAW, Emory University School of Medicine, Atlanta, Georgia
L. C. HYMES AND
Amer. J. Dis. Child., 135: 766-767 (Aug.) 1981 The authors report on 2 brothers from a nonendemic area for the hemolytic-uremic syndrome who suffered the syndrome 7 days apart. The 4-year-old index case presented with bloody diarrhea, vomiting and lethargy, and was found to have an elevated blood urea nitrogen and creatinine, low hematocrit and platelet count and blood smear with schistocytes. He was never hypertensive nor anuric and remained neurologically normal. Convalescence was uneventful. The 15-month-old brother suffered bloody diarrhea and vomiting 7 days after his brother's onset of symptoms. Laboratory values were similar. However, this child was anuric but normotensive. He had a grand mal seizure and respiratory arrest. Despite peritoneal dialysis the neurologic status deteriorated and the boy died. Postmortem evaluation showed renal glomerular thrombosis and extensive cortical necrosis. The brain showed areas of necrosis and edema without evidence of intravascular coagulation. The clinical course of these brothers did not conform to the pattern usually seen in siblings. The onset of illness <1 week apart is more typical of an endemic area and a brief interval generally is associated with a favorable prognosis. The authors indicate the need for close surveillance of potentially affected siblings for evidence of hemolytic-uremic syndrome. H.M.S. 10 references
A Rationale for the Routine Use of Transverse Abdominal Incisions in Infants and Children L. GAUDERER, Division of Pediatric Surgery, Rainbow Babies and Children's Hospital, University Hospitals of
M. W.
Clear Cell Sarcoma of the Kidney in Children: A Distinct Entity M. CARCASSONE, C. RAYBAUD AND G. LEBREUIL, Departments of Paediatrics and Paediatric Oncology, and Laboratory of Paediatric Pathology, School of Medicine, University of Marseille, Marseille, France J. Ped. Surg., suppl. 1, 16: 645-648 (Aug.) 1981 The authors report 4 cases of a type of renal tumor in children, which they advocate should be treated as a disease entity separate from the Wilms tumor group. The clear cell sarcoma makes up 2 to 5 per cent of renal tumors, occurs mainly in boys and has an age incidence similar to conventional Wilms tumor. Blastema tissue is not seen. Tumor cells are 10 to 20 µ. in diameter and are pleomorphic, round, stellar and sometimes spindle-shaped. The main histologic features are related to the oval or polygonal clear cells. The nuclei are round or oval, with a dark membrane, finely dispersed chromatin and 1 pointshaped nucleoeus. Ultrastructured study confirms the undifferentiation of the sarcoma, with no tendency toward fibroblastic or smooth and striated muscular differentiation. If renal tubules are seen they are entrapped within the packed tumor cells and no immature features are noted. Tubules are not seen in metastases. The tumors are typically large with a high frequency of bony metastases and lymphatic involvement. The prognosis is poor, worse than Wilms tumor. Often, this tumor is resistant
PEDIATRIC UROLOGY
versity of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania
Cleveland; and Department of Biometry, Case Western Reserve University, School of Medicine, Cleveland, Ohio
Pediatrics,68:45-49 (July) 1981
J. Ped. Surg., suppl. 1, 16: 583-586 (Aug.) 1981
Fourteen children from 5 to 17 years old with meningomyelocele and significant fecal soiling were examined by 3-balloon anorectal manometry. Eight patients who demonstrated rectal sensation were selected for biofeedback conditioning in an effort to improve fecal continence. Since all children were shown to have absent external sphincter responses reflexively and on voluntary command improved continence depended on learning to increase rectal tone by contracting gluteal and related muscles. Of the 8 patients 4 had significant improvement in fecal continence (2 continent, 1 improved 90 per cent and 1 improved 75 per cent). N euromotor testing was not reliable in determining potential candidates for successful biofeedback training but no patient who responded well had a defect proximal to L5. Sensory awareness of rectal distension and ability to contract gluteal or related muscles were essential for this training to be effective. H.M.S. 2 tables, 8 references
The best incision is one that provides optimal exposure with minimal trauma, allows extension, provides greatest tensile strength and maximizes patient comfort and cosmetic end result. The exposure and the ability to extend the incision are related to the diameter or shape of the cavity under consideration. The authors correlated the topography of the abdominal wall with growth. There were 91 children studied. None was markedly obese or cachectic and none had an abdominal wall defect or abdominal distension. In full-term children, when compared to the standard growth curves, the slope of the measurements of the abdominal wall showed a much smaller slope. This was especially true of a measurement of the costoiliac difference when there was practically no growth from birth to 16 years old. In premature infants the same general trend was observed although the curves were different. The authors concluded that the younger the child the relatively larger the abdominal cavity and abdominal wall. Both gridiron and transverse abdominal incisions have been shown to be superior to vertical celiotomy in children and adults. Infants, premature newborns and small children have a disproportionately large abdomen and liver. The transverse diameter of the abdomen is more pronounced in infants so that the abdominal cavity has an ellipsoidal shape. Consequently, the space for extension of vertical incision is somewhat limited, while lateral extension newborns are more easily achieved. The low position of the umbilicus in infants places a transverse supraumbilical incision in the anatomical center of the abdomen. An additional advantage of this incision is that there is virtually no need for selfretaining retractors and less relaxation is needed for operative exposure. The cosmetic advantage of placing an incision in the skin tension lines is well recognized. G. W.K. 4 figures, 23 references
Editorial comment. This seems a useful technique. Manometrics quickly identify the patients who can contract the sphincters somewhat and half of these benefited from training to a quite useful degree. L.R.K. Hemolytic-Uremic Syndrome in Two Siblings From a Nonendemic Area B. L. WARSHAW, Emory University School of Medicine, Atlanta, Georgia
L. C. HYMES AND
Amer. J. Dis. Child., 135: 766-767 (Aug.) 1981 The authors report on 2 brothers from a nonendemic area for the hemolytic-uremic syndrome who suffered the syndrome 7 days apart. The 4-year-old index case presented with bloody diarrhea, vomiting and lethargy, and was found to have an elevated blood urea nitrogen and creatinine, low hematocrit and platelet count and blood smear with schistocytes. He was never hypertensive nor anuric and remained neurologically normal. Convalescence was uneventful. The 15-month-old brother suffered bloody diarrhea and vomiting 7 days after his brother's onset of symptoms. Laboratory values were similar. However, this child was anuric but normotensive. He had a grand mal seizure and respiratory arrest. Despite peritoneal dialysis the neurologic status deteriorated and the boy died. Postmortem evaluation showed renal glomerular thrombosis and extensive cortical necrosis. The brain showed areas of necrosis and edema without evidence of intravascular coagulation. The clinical course of these brothers did not conform to the pattern usually seen in siblings. The onset of illness <1 week apart is more typical of an endemic area and a brief interval generally is associated with a favorable prognosis. The authors indicate the need for close surveillance of potentially affected siblings for evidence of hemolytic-uremic syndrome. H.M.S. 10 references
A Rationale for the Routine Use of Transverse Abdominal Incisions in Infants and Children L. GAUDERER, Division of Pediatric Surgery, Rainbow Babies and Children's Hospital, University Hospitals of
M. W.
Clear Cell Sarcoma of the Kidney in Children: A Distinct Entity M. CARCASSONE, C. RAYBAUD AND G. LEBREUIL, Departments of Paediatrics and Paediatric Oncology, and Laboratory of Paediatric Pathology, School of Medicine, University of Marseille, Marseille, France J. Ped. Surg., suppl. 1, 16: 645-648 (Aug.) 1981 The authors report 4 cases of a type of renal tumor in children, which they advocate should be treated as a disease entity separate from the Wilms tumor group. The clear cell sarcoma makes up 2 to 5 per cent of renal tumors, occurs mainly in boys and has an age incidence similar to conventional Wilms tumor. Blastema tissue is not seen. Tumor cells are 10 to 20 µ. in diameter and are pleomorphic, round, stellar and sometimes spindle-shaped. The main histologic features are related to the oval or polygonal clear cells. The nuclei are round or oval, with a dark membrane, finely dispersed chromatin and 1 pointshaped nucleoeus. Ultrastructured study confirms the undifferentiation of the sarcoma, with no tendency toward fibroblastic or smooth and striated muscular differentiation. If renal tubules are seen they are entrapped within the packed tumor cells and no immature features are noted. Tubules are not seen in metastases. The tumors are typically large with a high frequency of bony metastases and lymphatic involvement. The prognosis is poor, worse than Wilms tumor. Often, this tumor is resistant