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B r i e f clinical and laboratory observations
al 1~and Waldo et aP have shown in separate patients that atrial muscle in PAS has a lowered absolute resting m e m b r a n e potential, making the tissue refractory to electrical stimulation, producing atrial inexcitability. The unusually slow escape rhythm, particularly in Patient 1, suggests a disease process extending into the junctional portion of the cardiac conduction system. Similar nonspecific electron microscopic changes seen in biopsy material from our patients have been demonstrated by Yoneda et al. TM These nonspecific changes suggest that no major structural abnormality is present in our patients. The fact that atrial activity has returned after a period of 4 to 5 years would also support the absence of any fixed morphologic abnormality. Previous reports of familial PAS have stressed the degenerative nature of the disorder. 4-7 However, return of atrial activity after several years of atrial standstill is rare. The nature of the myocardial abnormality in our patients remains uncertain. The natural history of the disorder has been similar in both siblings, and excluding the possibility of mutual exposure to some toxic or infectious agent, we speculate that the arrythmia is part of a familial illness. N o r m a l skeletal muscle biopsy, obtained from Patient 2, rules out the possibility of a familial general myopathic process. The neurologic complications in our patients were probably due to the limited reserve capacity of the heart at a fixed, slow ventricular rate. Both children have responded favorably to the implantation of permanent fixed rate pacemakers, and no evidence of systemic disease in either child has been apparent.
The Journal of Pediatrics December 1980
REFERENCES
1. Rosen KM, Rahimtoola SH, Gunner RM, and Lev M: Transient and persistent atrial standstill with His bundle lesions: electrophysiologic and pathologic correlations, Circulation 44:220, 1971. 2. Bloomfield DA, and Sinclair-Smith BC: Persistent atrial standstill, Am J Med 39:35, 1965. 3. Waldo AL, Vitikaineu K J, Kaiser GA, Bowman FO, and Maim JR: Atrial standstill secondary to atrial inexcitability (atrial quiescence): recognition and treatment following open heart surgery, Circulation 46:690, 1972. 4. Waters DD, Nutter DO, Hopkins LC, and Dorney ER: Cardiac features of an unusual x-linked humeroperoneal neuromuscular disease, N Engl J Med 293:1017, 1975. 5. Harrison WH, and Derrick JR: Atrial standstill, Angiology 20:610, 1969. 6. Allensworth DC, Rice GJ, and Low GW: Persistent atrial standstill in a family with myocardial disease, Am J Med 47:775, 1969. 7. Williams DO, Jones EL, Nagle RE, and Smith BS: Familial atrial cardiomyopathy with heart block, Q J Med 41:491, 1972. 8. Cancilla PA, and Verity MA: Histochemical fiber types in clinical disorders of muscle, in Pearson CM and Mostefi FK, editors: The striated muscle, Baltimore, 1973, The Williams & Wilkins Company, p 77. 9. Nordenberg A, Vaghese PJ, and Nugent EW: Spectrum of sinus node dysfunction in two siblings, Am Heart J 91:507, 1976. 10. Yoneda S, Murata M, Veda A, Sato M, Takahashi M, Ito T, Sada T, Matsumoto S, Ito Y, Furuta S, Kamiyama A, and Akaike A: Persistent atrial standstill developed in a patient with rheumatic heart disease: Electrophysiological and histological study, Clin Cardiol 1:43, 1978.
The detection of acute gastrointestinal bleeding using in vivo technetium 99m pertechnetate-labeled erythrocytes Eduardo J. Rift, M.D.,* Patricia W. Hayden, M.D., and John K. Stevenson, M.D., Seattle, Wash.
TECHNETIUM 99M PERTECHNETATE has been used for the scintigraphic demonstration of Meckel diverticulum since its introduction in 1970 by Jewett et al. 1 Recently H e y m a n et al ~ used this technique to localize a vascular tumor in the small intestine during the flow
From the Departments of Pediatrics, Radiology, and Surgery, University of Washington School of Medicine and The Children's Orthopedic Hospital and Medical Center. *Reprint address: Children's Orthopedic Hospital and Medical Center, 4800 Sand Point Way N.E., Seattle, WA 98105.
phase of an abdominal pertechnetate scan by using in vivo ""~'Tc-labeled erythrocytes as an intravascular marker. Wingelberg et aP demonstrated the sensitivity of this method in detecting active gastrointestinal bleeding. We
Abbreviations used HUS: hemolytic-uremic syndrome RBC: red blood cell " have recently used the in vivo labeling method to localize the site of active bleeding in the colon of a 3-year-old boy with hemolytic-uremic syndrome.
0022-3476/80/120956 + 03500.30/0 9 1980 The C. V. Mosby Co.
Volume 97 Number 6
Brief clinical and laboratory observations
957
CASE REPORT
The patient was a 3-year-old white boy, in good health until approximately six days prior to admission when he developed a low-grade fever, vomiting, and foul-smelling diarrhea. Over the next 48 hours the diarrhea worsened and became bloody. He was treated with Lomotil and ampicillin, but the vomiting and diarrhea persisted and necessitated admission to another hospital for rehydration. On the second hospital day urine output diminished, the patient had a grand mal seizure, and he became obtunded; he was then transferred to The Children's Orthopedic Hospital and Medical Center. Physical examination revealed a distended abdomen with a fluid wave. The liver edge was palpable 2 cm below the right costal margin. The child was obtunded, responding only to noxious stimuli; cranial nerves were intact and results of the funduscopic examination were normal. Initial laboratory values included: Platelet count 67,000/mm :', hematocrit 34%, white blood count 19,770/mm ~ with 39% polymorphonuclears and 36% bands. RBC morphology was remarkable for fragments. Electrolytes: Na 115 mEq/1, K 3.8 mEq/l, CI 83 mEq/l, HCO~ 15 mEq/l. BUN was 50 mg/dl, and creatinine concentration 3.3 mg/dl. Coagulation screen was normal. A diagnosis o f HUS was made and peritoneal dialysis was started. On the eighth hospital day the dialysate fluid grew gram-negative rods, later identified as Bacteroides fragilis. On the ninth hospital day, he developed more abdominal distention, and roentgenograms showed markedly dilated transverse colon. After exploratory laparotomy a 15 cm segment of infarcted sigmoid colon was resected and a colostomy established; the remaining 10 cm of distal colonic segment was closed as a Hartman pouch. On the nineteenth hospital day the patient became hypotensive and began passing large amounts of bright red blood per colostomy, requiring administration of both blood and crystalloid to support his blood pressure. An abdominal roentgenogram showed a nonspecific gas patter u. A radionuclide flow study was proposed to localize the site o f intestinal bleeding. Thirty minutes following intravenous administration of 0.5 mg stannous chloride, the patient was positioned supine beneath a gamma camera and 5 millicuries of ~mTc pertechnetate injected into a femoral cannula. Continuous one-second abdominal images were acquired on magnetic tape and observed on a videoscope. The Figure shows selected 10-second integrated images from the first three minutes of the study. During the arterial flow phase a focus of radioactivity was seen in the fight upper quadrant below the liver image. As red cells were labeled the entire transverse colon became apparent and the labeled blood rapidly accumulated in the colostomy bag. This was interpreted as hemorrhagic colitis primarily involving the transverse colon. Because of the continued uncontrolled bleeding, the remaining proximal colon was resected and the terminal ileum was exteriorized and matured as a temporary ileostomy. Postoperatively the child did well, and over the next two months renal function returned. The patient was send home with an ileostomy. Two months after discharge, he was readmitted for closure of the
*J~.d
K.r-r ~
,J,,.d
t 7;~,
O,.)- J ' l " ~ Figure. Selected 10-second images show colonic bleeding: first m the region of the hepatic flexure (arrow 3-13s) then along the entire transverse colon (arrow 103-114s). The colostomy bag has been reflected toward the left upper quadrant and shows accumulated labeled blood (arrow 25-34s).
ileostomy and establishment of intestinal continuity. When seen in follow-up clinic he was continent and having two soft, formed stools per day. PATHOLOGY
REPORT
T h e serosal a s p e c t o f the c o l o n d e m o n s t r a t e d v a s c u l a r c o n g e s t i o n a n d h e m o r r h a g e to a m i l d d e g r e e , a n d the a s c e n d i n g a n d t r a n s v e r s e c o l o n was filled with b l o o d . T h e r e was m u l t i f o c a l i s c h e m i c necrosis o f m u c o s a a n d s u b m u c o s a . N e a r the h e p a t i c flexure t h e r e was circt referential m u c o s a l a n d s u b m u c o s a l u l c e r a t i o n with c o n g e s t e d g r a n u l a t i o n tissue.
958
B r i e f clinical and laboratory observations
DISSCUSSION In approximately 89% of patients with H U S there is a prodromal history of acute gastroenteritis. ~ It has been our experience that the acute gastroenteritis frequently progresses to bloody diarrhea and ischemic colitis, heralding the onset of acute renal failure. Hemorrhagic colitis, as manifested by hematochezia, falling hematocrit, and unstable vital signs, is not u n c o m m o n and usually requires surgical intervention. Localizing the site of active bleeding prior to surgery may m e a n the difference between a total or a subtotal colectomy. Since patients who develop this complication frequently have significant angiopathic coagulopathy, angiographic examination may be risky. In vivo~'~'mTclabeling of erythrocytes is a simple, noninvasive procedure that has advantages over other tracer techniques in detecting the presence of active gastrointestinal bleeding. Since over 95% of the ~mTc binds to RBCs, the radionuclide remains confined to the intravascular space, thereby reducing background activity and gastric secretion. The six-hour half-life of ~"~ is sufficiently long to allow delayed imaging if necessary, and yet is sufficiently short to minimize radiation exposure. The
The Journal of Pediatrics December 1980
whole body radiation dose from in vivo labeled RBCs is less than 0.020 rad per millicurie '"mTc, which is much less than the 1 to 5 rad skin dose from contrast arteriography. REFERENCES
1. Jewett TC, Dusgynski DO, and Allen JE: The visualization of Meckel's diverticulum with Tc-99m pertechnetate, Surgery 68:567, 1970. 2. Heyman S, Sacho B, Khettry J, and Steer M: Localization of bleeding small intestinal lesions using scanning techniques, Surgery 85:372, 1979. 3. Wingelberg GG, McKusick A, Strauss W, Waltman AC, and Greenfield AJ: Evaluation of gastrointestinal bleeding by red blood cells labeled in vivo with technetium-99m, J Nucl Med 20:1108, 1979. 4. Kaplan BS, Thompson PD, and deChaparevian JP: The hemolytic-uremic syndrome, Pediatr Clin North Am 23:761, 1976. 5. Riella MC, George CR, Hickman RO, Striker, GE, Slichter, SJ, Narker L, and Quadracci CJ: Renal microangiopathy of the hemolytic-uremic syndrome in childhood, Nephrology 17:18B, 1976. 6. Lieberman E: Hemolytic-uremic syndrome, J PEDIATR80: I, 1972.
Percutaneous transluminal renal artery angioplasty in a
31/2-year-oM hypertensive girl Thomas A. McCook, M.D., Steven R. Mills, M.D.,* Donald R. Kirks, M.D., Dennis K. Heaston, M.D., Hiiliard F. Seigler, M.D., Robert B. Malone, P.A., and Stephen G. Osofsky, M.D., Durham, N. C.
SINCE the original description of transluminal balloon angioplasty by Dotter and Judkins 1 in 1964, extensive experience in adults has proven this technique to be both safe and effectiveY * Although the vast majority of transluminal angioplasties have been performed for peripheral occlusive atherosclerotic disease, recent reports have described the successful treatment of renovascular hypertension by percutaneous balloon dilatation. Percutaneous transluminal renal artery angioplasty has been successful in correcting renal artery stenosis secondary to atherosclerosis and fibromuscular dysplasia in adults?' 6 We present the first reported case of successful transluminal balloon dilatation of renal artery stenosis in a pediatric patient.
From the Departments of Radiology, Pediatrics, and Surgery, Duke University Medical Center. *Reprint address: Department of Radiology, Box 3808, Duke University Medical Center, Durham, N. C. 27710.
CASE REPORT
A 389 girl was noted on routine physical examination to have a blood pressure of 135/88 mm Hg. Subsequent repeat measurements in all extremities ranged from 135 to 150 mm Hg systolic with diastolic values from 86 to 105 mm Hg. There was no history of headaches, visual disturbance, vomiting, flushing, Abbreviation used PTRA: percutaneous transluminal renal artery angioplasty sweating, diarrhea, or urinary tract symptoms. Past history was negative except for asthma, for which she was receiving no medication. There was no history of hypertension in the immediate family. On admission the blood pressure in the sitting position was 155/122 mm Hg in the right arm. with similarly elevated values in the left arm and lower extremities, The remainder of the physical examination was normal; in particular, no stigmata of neurofibromatosis were noted. Laboratory studies including urinalysis, blood count, serum electrolytes, BUN, creatinine,
0022-3476/80/120958 +03500.30/0 9 1980 The C. V. Mosby Co.