Resolution of renal amyloidosis.

A patient with renal amyloidosis and the nephrotic syndrome consequent to extensive infected burns demonstrated both clinical resolution of the nephrotic syndrome and morphologic regression of the renal amyloid deposits over a six year period. The regression of the amyloid deposits was associated with several changes in the glomerular capillary wall resulting in a double capillary wall contour. This case indicates that deposits of amyloid in the kidney may regress and suggests a sequence of events in this resolution.

Growth retardation in children with ulcerative colitis: the effect of medical and surgical therapy.

The growth of 37 children with ulcerative colitis have been analyzed. While conventional growth charts showed only percentile changes in height, height data plotted on Tanner et al.'s growth charts showed increases and decreases in growth velocity. Growth retardation is a prominent complication of ulcerative colitis with onset on bowel symptoms. Both ulcerative colitis and "high-dose" steroid therapy (greater than 12 mg/sq m/day of cortisol) can hinder growth but in some instances there is a growth spurt after high-dose steroid therapy. "Low-dose" steroid therapy does not retard growth. Colectomy is more effective than high-dose steroid therapy in reversing the growth retardation caused by ulcerative colitis and is of greatest value if not delayed too long. Growth following subtotal colectomy with ileorectal anastomosis (Aylett procedure) is not likely to be as much as that after subtotal colectomy with ileostomy. Growth retardation is infrequently the only indication for surgical intervention but ileostomy and colectomy are appropriate for this complication of ulcertive colitis in itself when not improved by adequate medical treatment.

Posterior mediastinal teratoma (cystic dermoid): diagnosis by computerized tomography.

A patient with posterior mediastinal dermoid cyst is presented. With computerized tomography of the mediastinum, it was noted that the mass had attenuation values consistent with sebaceous material and fatty tissue and the diagnosis was proven at surgery and pathologically.

Pediatrics at Mount Sinai.

The Department of Pediatrics at The Mount Sinai Hospital was founded more than 120 years ago. During the ensuing years it had five outstanding leaders, Drs. Abraham Jacobi, Henry Koplik, Bela Schick, Horace Hodes, and Kurt Hirschhorn. The evolution of its beginning as a "small" clinic to its present position as one of the outstanding pediatric centers in this country reflects its interest in and devotion to the care of children.

Pediatric gastroenterology at the Mount Sinai hospital.

The history of pediatric gastroenterology at Mount Sinai begins in 1960. Early publications by Drs. Korelitz and Gribetz on the management of inflammatory bowel disease in children served as the preface to forty years of progress in this important area. The history of pediatric gastroenterology includes important work by many individuals, including Horace Hodes, Lotte Strauss and Frederick Kopel. Early observations on the nature of inflammatory bowel disease (IBD), and its course, preceded work on nutritional therapies for IBD, mechanisms of gene-nutrient interactions, regulation of gene transcription, and molecular processes involved in bile transport in the liver and small intestine. Over the last twenty years, the division has grown in size and reputation. Today there are fourteen full-time faculty - 9 M.D.'s and 5 Ph.D.'s - who work in three funded research laboratories. There are also five advanced practice nurses (including three nurse practitioners), two social workers and two nutritionists, as well as several administrators and assistants. In addition to being recognized as a premier center for the treatment of children with general pediatric gastroenterological problems, especially inflammatory bowel disease, the division is also known as one of the nation's largest pediatric liver and liver transplant centers, and it is rapidly becoming one of the largest pediatric short gut syndrome and small bowel transplant centers.

Ileal duplication cyst: the importance of sodium pertechnetate Tc 99m scanning.

Duplications of the alimentary tract may cause extensive bleeding when ectopic gastric mucosa is present. A successful diagnosis can be made preoperatively with the use of conventional barium studies as well as sodium pertechnetate Tc 99m scans.

Noninvasive estimation of serum bilirubin.

OBJECTIVE: The aim of this study was to evaluate the accuracy and clinical usefulness of a new computer-driven, hand-held device (Chromatics Colormate III) to estimate serum bilirubin from skin-reflectance (skin color) of neonates. STUDY DESIGN: A total of 2441 infants (both term and premature) at two hospitals had repeated measurements of transcutaneous bilirubin. Of these infants, 900 had one or more laboratory determinations of serum bilirubin. Many of the infants had early measurements of skin color before the onset of jaundice. A visual estimate of the degree of jaundice was made by a health care worker when the laboratory study was drawn. A subgroup of 61 infants was also studied while undergoing phototherapy with a total of 284 comparisons obtained. The reproducibility of the instrument was assessed separately using standardized color tiles and repeated measurements by multiple operators. RESULTS: The range of serum bilirubin measurements that had concurrent skin color measurements was 3.22 to 338.1 micromol/L (0.2 to 21 mg/dL). The linear regression indicated an r = 0.956, and 95% of the skin color measurements were within 32.2 micromol/L (2.0 mg/dL). There was no interference with the accuracy of the device because of infant race or weight, or because of the use of phototherapy. The device provided reproducible information when infants were tested repeatedly over 30 minutes; the coefficient of variation for the transcutaneous bilirubin measurement was 3.1% around a mean estimate of 135.32 micromol/L (8.4 mg/dL). CONCLUSION: The Chromatics Colormate III allows for a clinically useful estimate of serum bilirubin in a wide variety of infants. By using a color discrimination algorithm and obtaining a skin measurement before the onset of icterus, this instrument can provide valuable clinical information that obviates the need for serum bilirubin determinations. Its use in newborn nurseries may allow physicians to shorten length of stay more safely and decrease the use of invasive blood tests.

Trisomy 13 and Rubinstein-Taybi syndrome.

Initial diagnosis of Rubinstein-Taybi syndrome was made in an infant with a prominent nose and broad thumbs and first toes. However, due to the presence of other anomalies such as low-set, malformed ears, anti-mongoloid slant of the eyes, colobomata of the iris, and cleft palate, cytogenetic studies were carried out and the diagnosis of trisomy 13 was confirmed. Since, occasionally, trisomy 13 syndrome may mimic the Rubinstein-Taybi syndrome, cytogenetic studies should be considered in all patients with clinical diagnosis of Rubinstein-Taybi syndrome.