Biomedical subjects
Publications and source records attributed to S Variend.
Carboxyhaemoglobin measured in 50 infant deaths showed no significant difference between home and hospital deaths nor between explained and unexplained deaths. Carbon monoxide toxicity is unlikely to have an important role in the pathogenesis of sudden infant deaths. The generally low carboxyhaemoglobin concentrations are probably due to endogenous production.
Fat necrosis was present in 22 of 400 cases of consecutive postperinatal mortalities investigated to assess the presence and pattern of deep fat necrosis. In just over 50% of the cases of fat necrosis the cause of death was categorised as sudden infant death syndrome, which also showed more severe degrees of necrosis. The mechanism of necrosis may be vascular hypoperfusion, possibly related to shock, and brown adipose tissue, on account of its high metabolic activity and rich capillary plexus, may be particularly vulnerable to infarction. The occurrence of fat necrosis in association with other causes of death did not provide any definite clue as to the nature of the alleged shock.
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Recurrent apnoea and chronic hypoventilation have been implicated in the pathogenesis of the sudden infant death syndrome (SIDS) and markers of chronic hypoxaemia have been reported in such infants at post mortem examination. Markers of chronic hypoxaemia are common in cyanotic congenital heart disease. Glomerular enlargement in congenital heart disease is said to be related to hypoxaemia although the precise mechanism whereby this occurs is not clear. We have established a normal range of glomerular size for the postperinatal period and confirmed glomerular enlargement to be a common finding in children with congenital heart disease of similar age. In contrast glomerular size in SIDS is not different from controls. The results question the role of significant chronic hypoxaemia being involved in these deaths.
Persistence of the proximal portion of the right umbilical vein is described in a premature infant. Infracardiac total anomalous pulmonary venous connexion and unilateral renal agenesis with ipsilateral phocomelia and unicornuate uterus were associated anomalies. The mother had received carbamazepine therapy for epilepsy. Previously reported cases of persistent proximal right umbilical vein have shown a variety of other malformations but a single umbilical artery has been the most consistent.
Four infants, apparently thriving and without clinical evidence of disease, died suddenly at ages ranging from 2 to 6 months. Inclusions bearing cells pathognomonic of cytomegalovirus infection were shown microscopically in a small number of extraneural organs. In view of the lack of associated tissue destruction on microscopy and the apparent well being of the infants before death whether the function of these organs had been impaired to any important degree was questionable: such limited disease, consequently, could not have contributed substantially to the cause of death. The brainstem, on the other hand, consistently showed small numbers of glial nodules. Damage to strategically located neurones associated potentially with the organisation of vital function was a possible basis of sudden death. Alternatively, the small number of glial nodules may have represented a residue of previous more severe brainstem disease, which had possibly started while the baby was in the uterus.
Two hundred postmortems were studied retrospectively to assess the frequency of nuclear fragmentation (NF) and epithelioid change (EC) in the germinal centers of lymphoid tissues in children. Sections of spleen, mesenteric lymph node, tonsils, and appendix were included in the survey. Thirty-seven of 200 cases (18.5%) showed NF or EC or a combination. These morphological changes were considered to represent different stages of a common pathological reaction for which a classification is proposed. The type of lymphoid change correlated approximately with the duration of the preceding illness. Although more common between the ages of 1 and 3 years, they were found to extend throughout the age range studied. There was a negative correlation between the presence of NF and EC and the cause of death, but a significant association with clinical documentation of shock was present, supporting previous evidence that shock is the significant factor in pathogenesis.
Two hundred consecutive cases of the sudden infant death syndrome were reviewed for the presence of fat in the liver; 14 showed diffuse panlobular microvesicular fatty change indistinguishable from that found in Reye's syndrome. Samples of frozen liver were available in five of the 14 cases; histochemical analysis showed well preserved cytochrome oxidase and succinate dehydrogenase activity in all five, uncharacteristic of Reye's syndrome. Fatty acyl-coenzyme A dehydrogenase activity in the liver was assayed biochemically in two of the same five cases with severe hepatic fatty infiltration; both showed a defect in medium chain acyl-coenzyme A dehydrogenase activity using the substrate octanoyl-coenzyme A. Both cases also showed cerebral oedema in association with fatty infiltration of renal tubules, myocardium, and skeletal muscle, characteristic of Reye's syndrome. It is concluded that diffuse panlobular microvesicular fatty change of the liver in victims of the sudden infant death syndrome, although essentially non-specific, indicates that the state of mitochondrial enzymes should be investigated.
A nine-year-old girl was fortuitously found to have a symptomless metastatic islet cell tumour (apudoma) of the head of the pancreas, the first to be described in a child. The tumour has not changed in size over 3 years and the patient has shown normal growth and development. Initial chemotherapy had no effect.
The past decade has seen significant advances in the treatment of childhood malignancies accompanied by appreciable improvement in survival rates. Treatment programmes have been largely formulated to meet the specific characteristics of individual tumours, as well as being based on the extent of disease presented at diagnosis. In selecting the most appropriate treatment protocol, accurate histological categorization of resected or biopsied tumour is thus of paramount importance. In the paediatric age range in which so many tumours lack differentiation as to present as, or mimic, other small cell tumours, routine methods are often insufficient to resolve problematic histology. A wide range of special techniques is now at hand to assist the pathologist with this problem and this review is an attempt partly to rationalize the application of available methodology. Of considerable importance also is a knowledge of the behavioural characteristics of this group of tumours, their prototypic histology, as well as the range of morphological variability.
Forty-nine Spitz nevi occurring in children were reviewed and sampled extensively in order to assess the incidence of vascular invasion. Evidence of vascular invasion was found in seven (14.3%) cases. The endothelium of such vessels was negative on immunoperoxidase staining for Factor VIII-related antigen suggesting the nevus cells to be in lymphatic channels and not blood vessels. No unusual histological or clinical features characterized the group. All patients are alive and well some years after local excision therapy. It is concluded that lymphatic invasion by nevus cells in Spitz nevi is not uncommon and its presence should not tempt the pathologist into a diagnosis of melanoma.
Six rhabdomyosarcomas were assessed by means of a battery of enzyme histochemical methods. The reactions were compared with those of a small number of other tumours belonging to the small-cell tumour category. Four of the rhabdomyosarcomas were positive for myophosphorylase and acetylcholinesterase. Myoblasts were strongly reactive for adenosine triphosphatase at alkaline pH and after acid pre-incubation, whereas the small undifferentiated neoplastic cell of the four alveolar rhabdomyosarcomas showed also discernible cytoplasmic reaction, but only after acid pre-incubation. Other tumour categories revealed positive staining for adenosine triphosphatase with acid pre-incubation but the degree of reaction was minimal by comparison. Other enzyme reactions were variable and, generally, did not distinguish between different tumour categories. It is concluded that enzyme histochemistry has a potential role in the diagnostic evaluation of the small cell tumour and should be included in the growing list of special techniques that may assist the pathologist confronted with this problem.
A comparative study of beta enolase and myoglobin as markers of muscle differentiation in rhabdomyosarcoma was carried out, using an immunoperoxidase peroxidase antiperoxidase technique. Material from 26 cases of childhood rhabdomyosarcoma was studied and subdivided into embryonal and alveolar types. Positive cytoplasmic staining for beta enolase was seen in 85% of tumours studied (91% alveolar, 79% embryonal), whereas positive staining for myoglobin was detected in only 69% of tumours (82% alveolar, 64% embryonal). beta Enolase and myoglobin are useful in the histological diagnosis of rhabdomyosarcoma, and of the two, beta enolase seems to be the more sensitive.
A quantitative and morphological study was made of the corpus callosum in children to assess possible sex differences. The shape of the splenium of the corpus callosum was observed to vary between bulbous and cylindrical types with respect to the body of the callosum. The variation was not gender-specific and covariate analyses taking account of brain weight and age detected no differences in splenial width or area between males and females in childhood. This is in contrast to the situation reported in adults.
A child with bilateral Wilms' tumors is reported. The left renal tumor showed nephroblastoma with several tissues of apparent mesenchymal derivation and tubules with diverse epithelial differentiation. The right-sided tumor showed the more familiar triphasic pattern of nephroblastoma. Initial percutaneous renal biopsy of the left tumor did not reveal nephroblastoma but showed tubules with various epithelia and mesenchymal elements, and led to a diagnosis of teratoma. The pathogenesis of this complex neoplasm is discussed, and argument is presented that primitive renal blastema may be capable of more diverse differentiation than has previously been realized. A comparison is drawn between this neoplasm and some cases of hepatoblastoma. Certain cases reported as renal teratoma may be similar in nature.
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Symptomatic ovarian cysts in newborn are rare. In a recent series of cases of congenital ascites ( Griscom et al 1977) only one case out of 27 was caused by an ovarian cyst. We describe a case in which congenital ascites was caused by a ruptured corpus luteum ovarian cyst.
Seven unexplained, protracted infant deaths with similar clinical, biochemical and laboratory characteristics are described, being similar to other groups reported previously. Although autopsy examination frequently revealed extensive tissue damage, a primary cause of death could not be elucidated. It is argued that this condition represents a protracted form of conventional cot death and should be considered in any severely shocked infant where the cause is not readily identifiable. Many of the clinical and pathological manifestations are similar to those described in endotoxic shock and a small intestinal mucosal abnormality on histology of unknown pathogenesis was common.