Roma Chandra

EVALUATION OF 99MTECHNETIUM-DIMERCAPTO-SUCCINIC ACID RENAL SCANS IN EXPERIMENTAL ACUTE PYELONEPHRITIS IN PIGLETS

We evaluated the sensitivity and specificity of 99mtechnetium-dimercapto-succinic acid renal scans in the detection and localization of experimental acute pyelonephritis in piglets. To create pyelonephritis vesicoureteral reflux of infected urine was surgically induced in 22 piglets. Nine animals were evaluated with a dimercapto-succinic acid renal scan at 1 week and 13 at 2 weeks. Autopsy and histopathological examination of the kidneys were performed subsequently. The location and extent of the inflammatory response found on histopathological examination were compared to dimercapto-succinic acid renal scan findings in a blinded fashion. Of the 22 kidneys subjected to vesicoureteral reflux 15 had positive histopathological findings of acute pyelonephritis, including 13 detected by the dimercapto-succinic acid renal scan findings. The 2 kidneys in which inflammation was not detected had only minimal grade I lesions and were grossly normal. There were no false positive scans in any of these kidneys. The sensitivity of the scan for detection of acute pyelonephritis in the kidneys subjected to reflux was 87 per cent and the specificity was 100 per cent. Furthermore, in these same kidneys the dimercapto-succinic acid renal scan findings correctly predicted the presence or absence of individual pyelonephritic lesions in 62 of 66 poles for an over-all agreement rate of 94 per cent. Thus, dimercapto-succinic acid renal cortical imaging is a highly sensitive and reliable imaging modality to detect and localize experimental acute pyelonephritis in piglets.

Ductal remnants in extrahepatic biliary atresia: A histopathologic study with clinical correlation.

Atretic ducts were studied in 34 infants undergoing the portoenterostomy procedure for biliary atresia. The specimens were grouped into three categories on the basis of the size of the lumen at the proximal margin: Type I with a lumen 150 mu or greater, Type II with single to multiple ductal structures measuring less than 150 mu, and Type III with no identifiable epithelium-lined structures in fibrous connective tissue. A significant correlation existed between postoperative bile drainage and the duct type; bile drainage occurred in all five patients with Type I ducts, 18 of 21 patients with Type II ducts, and one of eight patients with Type III ducts. Furthermore, the duct type was the only feature that correlated with the ultimate outcome. Ductal inflammation and hepatic histology did not correlate with postoperative bile drainage or clinical course.

Aortic Valve Atresia: A New Classification Based on Necropsy Study of 73 Cases

Certain clinical and morphologic observations are described in 73 necropsy patients with aortic valve atresia. The mean age at death was 5 days; 80 percent died during the first week of life, and 70 percent were boys. Of the 73 patients, 69 (95 percent) had a hypoplastic left ventricle with intact ventricular septum and either an atretic (25 patients) or hypoplastic (44 patients) mitral valve. The other four patients had a well developed left ventricle with one or more defects in the ventricular septum and either an atretic (one patient) or well developed (three patients) mitral valve. Review of previous reports on aortic valve atresia disclosed that a well developed left ventricle or ventricular septal defect in association with absence of the aortic valve was extremely rare. A new classification for aortic valve atresia is presented based on the status of the ventricular septum, which in turn appears to determine the size of the left ventricular cavity. The predilection for male subjects for all types of aortic valve disease, including atresia, is emphasized.

Value of endomyocardial biopsy in infants, children and adolescents with dilated or hypertrophic cardiomyopathy and myocarditis

Endomyocardial biopsy was performed in 20 symptomatic pediatric patients with the following clinical diagnoses: dilated cardiomyopathy in 16, hypertrophic cardiomyopathy in 2 and myocarditis in 2. Transfemoral biopsy was performed without complications in patients aged 2 months to 16 years; 6 were less than 1 year old. An average of five right ventricular specimens were obtained for histologic and ultrastructure study from each patient. In 16 patients with the clinical diagnosis of dilated cardiomyopathy, biopsy findings were compatible with the diagnosis in 8, but changed the diagnosis as follows in the other 8: myocarditis, 4; hypertrophic cardiomyopathy, 2 and carnitine deficiency syndromes, 2. In two patients with the clinical diagnosis of hypertrophic cardiomyopathy, biopsy findings confirmed the diagnosis in one and were normal in the other who had an encapsulated cardiac fibroma at operation. In two patients with the clinical diagnosis of myocarditis, biopsy findings confirmed the diagnosis in one and suggested dilated cardiomyopathy in the other. In the entire series, 25% had biopsy evidence of inflammatory disease. Biopsy findings were confirmed at subsequent autopsy in five cases.

Immunologic parameters in histiocytosis-X

The immunocompetence of six infants with the clinical form of Letterer‐Siwe disease and seven older children in various phases of histiocytosis was evaluated. In most of the patients, delayed hypersensitivity, lymphocyte blastogenesis to mitogens and allogeneic cells, bactericidal killing, and leukocyte nitroblue tetrozolium dye reduction were found to be within normal limits. One patient with the infantile form of histiocytosis and one older child could not be sensitized to DNFB. The lymphocytes from two infants were hyporeactive to mitogeninduced stimulation. This reverted to normal after chemotherapy was instituted. Decreased immunoglobulin levels were found in two infants. Several patients exhibited elevated immunoglobulin levels. In general, the immunologic abnormalities improved following chemotherapy. No evidence of a combined immunodeficiency disorder was found in either group of patients. It is felt that the immunologic abnormalities which were found were either secondary to malignant cell replacement rather than an underlying cause of the disease.

Prepubertal male gynecomastia

Only 18 cases of prepubertal male gynecomastia are recorded in the medical literature; to these we add 5 more. The need to differentiate idiopathic gynecomastia from pathologically induced causes is discussed. The microscopic examination of the breast tissue in one of our patients showed cystic dilation of the ducts, a rarely described occurrence in prepubertal children.

Clinical, laboratory, and histologic observationsin euthyroid children and adolescents with goiters

Seventy-seven euthyroid children and adolescents with nontoxic goiters have been evaluated. Laboratory studies including thyroid function tests, measurement of thyroid antibodies, and biopsy of the thyroid gland were performed. Biopsy revealed that 43 of the patients had chronic lymphocytic thyroiditis, 28 had simple goiters, 4 had adenomatous goiters, and 2 had hyperplasia of the thyroid gland. The clinical and laboratory features used in the differential diagnosis of nontoxic goiters are discussed. Full replacement doses of thyroid hormone have been recommended as treatment in all patients with chronic lymphocytic thyroiditis and colloid goiters.

Small left atrium and change in contour of the ventricular septum in total anomalous pulmonary venous connection: A morphometric analysis of 22 infant hearts

Morphometric measurements of 22 hearts with total anomalous pulmonary venous connection (TAPVC) were compared with measurements of 8 matched control specimens without heart disease. Each of the TAPVC specimens had a shorter left atrium, smaller left atrial surface area and larger diameter of the fossa ovalis. In addition to increased length of the right ventricle and larger circumferences for tricuspid and pulmonary valve anuli, the left ventricular contour of the ventricular septum was flat or convex in 18 of the 22 hearts; the septum was significantly longer than normal in these specimens and wider at its midportion. Because mitral and aortic valve anuli were normal in circumference, the data suggest that left ventricular volume is not decreased despite change in ventricular shape.

Single ventricle with truncus arteriosus in siblings

Abstract Single ventricle and truncus arteriosus (TA) are relatively uncommon congenital cardiac defects. Keith et al. 1.2 reported these lesions to occur, respectively, in 1.1% and 0.4% of children with congenital cardiac defects. The occurrence of TA with a single left ventricle (LV) often has been theorized as a developmental stage but not previously described. This report describes siblings with the unique association of single LV with rudimentary right ventricular (RV) outflow chamber, TA type I, ostium primum atrial septal defect (ASD), and common atrioventricular (AV) valve.

Focal Giant Cell Cardiomyopathy with Beckwith-Wiedemann Syndrome

Cardiac involvement in Beckwith-Wiedemann syndrome is mostly limited to mild cardiomegaly. Although these patients have visceromegaly, macroglossia, gigantism, and adrenal cytomegaly, no significant myocardial changes have been described. An infant with dysmorphic features of this syndrome had supraventricular tachycardia since birth. Nodular lesions were present in the right atrium. Morphologically these lesions were composed of hypertrophic myocardial fibers admixed with multinucleated giant cells of myogenic origin. The exact nature of these lesions remains undetermined. It is postulated that hypertrophic myocardial cells may represent cardiac cytomegaly as a manifestation of the accelerated growth potential of cells seen with this syndrome.