H. Joachim Wigger

Thromboses due to catheterization in infants and children

A recent 3.5-fold increase in thrombosis of large vessels in autopsies is believed to be related to direct mechanical injury and alteration of flow in catheterized vessels. Although a useful diagnostic and therapeutic tool, the catheter may cause serious complications. In 75 per cent of the cases the “catheter thromboses” were accompanied by infarcts of major organs; in 12 per cent the sequelae of catheterization were considered to be the cause of death.

Necrotizing enterocolitis in term infants

Despite the fact that necrotizing enterocolitis is considered a disease of premature infants, 20% of all affected infants at Babies Hospital over the past 20 years were products of term gestations. Two distinct subgroups of such infants were noted (1) five infants with congenital heart disease and/or congestive heart failure (e.g.hypoplastic left heart syndrome), all but one of whom developed the disease in the first week of life; (2) eight infants who developed the disease at a much later age after a protracted period of diarrhea. This histopathologic features of the disease in term infants are the same as those in premature infants. Further, the pathogenesis of the disease in term infants does not appear to differ basically from that in premature infants. These facts, lead away from the concept of NEC as a disease of simple etiology.

High incidence of lung perforation by chest tube in neonatal pneumothorax

Lung perforation by chest tube was noted to be a frequent complication of percutaneous pleural drainage in the newborn Infant with pneumothorax. Between 1972 and 1976, 12 such perforations were noted at autopsy. Of 209 consecutive patients admitted to the Neonatal ICU with clinical RDS, 28 (13.4%) developed a pneumothorax requiring pleural drainage and 21 of these died. Twenty were autopsied and a lung perforation directly related to the pleural drainage was found in seven. None of these lesions had been diagnosed clinically. If the survivors were assumed to be free of this complication, the incidence of lung perforation following pleural drainage was 25%. The true clinical incidence in our patients with RDS can only be equal to or higher than that number. Iatrogenic lung perforation is likely to contribute to the fatal course of the underlying disease or, in survivors, probably prolongs the period of recovery. The high incidence of this complication suggests a need to re-evaluate the technique of pleural drainage in the newborn infant with pneumothorax.

Fatal laryngeal obstruction by iatrogenic subglottic cyst

Prolonged nasotracheal intubation in a 3 1/2-month-old premature infant produced obliteration of the laryngeal ventricle and scarring of vocal cord musculature, and also resulted in the development of subglottic retention cysts, one large enough to cause fatal laryngeal obstruction.

Calcified Portal Vein Thromboemboli in Newborn and Stillborn Infants

Calcified portal vein thromboemboli are not, as a rule, considered in the differential diagnosis of abdominal calcifications in the hepatic area in infants. A review of 13 autopsy cases with a retrospective examination of available chest and abdominal films showed that this entity could have been recognized in some instances (1). More recently, the roent-genographic diagnosis of calcified portal vein thrombi antemortem was suggested in 2 patients and verified at autopsy. This article presents the main clinical and radiological data from a series of 21 cases, 19 collected since 1954, representing 0.6 per cent of all perinatal and pediatric autopsies in this period. More detailed clinicopathological correlations, pathologic observations, and pathogenetic mechanisms will appear in a subsequent paper. Gross and Microscopic Anatomy The lesions may be single or multiple. They are usually subcapsular, appearing as nodules or as zig-zag or branching lines of a chalky-yellow color, and average less than 1 mm in wi...

Complete maturation of neuroblastoma with bone metastases in documented stages

Abstract Spontaneous regression of neuroblastoma, one of the most common tumors of childhood, is rare, occurring in 1–2% of cases. However, this lesion does have the highest rate of spontaneous regression of any human cancer. 1 This regression can occur as complete disappearance of the tumor or as maturation to a benign tumor. Partial or complete maturation has been reported, 2,3 most often in young infants, who had nonadrenal primary tumors and no osseous metastases. Everson and Cole reported that five of their 29 cases of spontaneous regression of neuroblastoma showed maturation to ganglioneuroma. 1 Among 170 patients with neuroblastoma or ganglioneuroblastoma seen at the Babies Hospital, we know of five patients whose tumor has matured to ganglioneuroma. One of these patients is reported here. She is unusual insofar as she showed documented maturation from neuroblastoma via ganglioneuroblastoma to benign ganglioneuroma in spite of initial exacerbation of the neoplastic disease and widespread bony metastases.

Angiotensin-converting enzyme: Effect of antienzyme antibody in vivo

Angiotensin-converting enzyme (EC 3.4.15.1) is a mammalian COOH-terminal dipeptidyl peptidase which catalyzes the release of His-Leu from angiotensin I to yield angiotensin II [ 11, the vasoactive agent of the renin-angiotensin system [2]. The same enzyme inactivates bradykinin, a vasodepressor nonapeptide, by cleavage of the COOH-terminal and adjacent dipeptides [3,4]. We have recently shown, using specific fluorescein-labeled antibody, that converting enzyme is present in the luminal cells of the vasculature of every organ examined, including lung, liver, kidney, pancreas, adrenal and spleen [5]. While extrapulmonary conversion [6-91 and converting activity [lO,l l] are well documented, the lung is thought to play an important role in both conversion of angiotensin I to II [ 12,131 and the degradation of bradykinin [ 141 in the circulation. This is because the lung is perfused by the entire circulating blood volume and has a very large capillary surface area. Further, enzymatically generated angiotensin II is not taken up by the lung [ 13,151 but delivered to the systemic circulation where it exerts its pressor effect. Recent immunohistochemical evidence has indicated that pulmonary converting enzyme may be localized on the luminal surface of the endothelial cells [ 161. The possibility that converting enzyme is accessible to circulating antibodies prompted us to examine the effects in vivo of antibody with anticatalytic activity.

Epidermoid cysts of the liver.

To the four recorded cases of epidermoid cysts of the liver are added two cases recently seen in children. One was in a 4-yr-old girl and associated with biliary cirrhosis. The other was in a 5-mo-old boy. The former was treated by roux-en-y cystjejunostomy, the latter by enucleation. The origin of these cysts is unknown. One theory suggests derivation from accessory foregut buds. Because of their malignant potential, treatment should be by excision. Where this is impossible, roux-en-y cystjejunostomy offers satisfactory palliation.

Effects of total parenteral nutrition and semi-starvation on the liver of beagle puppies☆

The effects on hepatic growth of fat-free TPN and semistarvation have been studied in newborn beagle puppies. Our data indicate that liver growth during TPN is characterized by normal deposition of protein and minerals, with increased glycogen and decreased lipid content. While the lipid pattern of TPN livers is normal, the phospholipid fraction contains an abnormal fatty acid pattern, consistent with the development of essential fatty acid deficiency. The exact physiologic significance of this is unknown, but it is tempting to speculate that changes in integrity of the hepatic cell membrane as a result of this abnormal fatty acid pattern might account for some of the clinical abnormalities of hepatic function during TPN.

Disseminated mycobacterial histiocytosis due to M. fortuitum associated with helper T-lymphocyte immune deficiency

Mycobacterial histiocytosis is a rare disease usually associated with haematological or immunological disorders. We report a fatal case caused by M. fortuitum infection showing the typical disseminated histiocytosis. Immunological investigations revealed impaired cellular immunity demonstrated by negative skin tests with different “recall-antigens”, and in vitro an isolated defect of helper T-lymphocytes in the peripheral blood which in combination with hypergammaglobulinemia suggests a “lymphocyte mal distribution syndrome”.