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Secondary hemophagocytic lymphohistiocytosis (hemophagocytic syndrome, massive macrophage activation syndrome) is a rare, aggressive, severe syndrome with a frequent fatal outcome. The pathogenesis of the syndrome, the role of viral infection, and the development of an excessive, aberrant, and inflammatory response leading to multiple organ dysfunction and sepsis remain unclear so far. The paper presents the data available in the literature and describes the authors' own observation of secondary hemophagocytic lymphohistiocytosis in a 14-year-old girl who had Epstein-Barr virus infection and died of severe multiple organ dysfunction. The in vivo diagnosis was based on the typical clinical presentations of the disease, the detection of the virus by a polymerase chain reaction assay, and the determination of blood ferritin levels. Contemporary morphological diagnostic criteria (immunohistochemical reactions with anti-EBV and anti-CD68 monoclonal antibodies; Epstein-Barr virus encoded RNA (EBER) in situ hybridization) are shown; and clinical and pathological comparisons have been made.

The data of renal ultrasonographic and Doppler studies were comparatively assessed in children with congenital hydronephrosis. The degree of renal B-mode ultrasound hemodynamic parameters was used as the basis for grouping of the children. The resistance index (RI) of all branches of renal arteries receives attention. Morphological studies were carried out on renal biopsy specimens from 29 children from different groups and on 12 removed kidneys; the expression of TGF beta1 and alphaSMA was revealed using the streptavidin-biotin-peroxidase method. Morphological changes as hypoplasia or dysplasia became more pronounced from Group 1 to Group 3, RI increasing to peak in Group 3. Vascular changes were confined to compensatory processes following the pattern of remodeling that was manifested by vascular wall thickening and a gradual increase in RI. Failing compensatory processes resulted in the development of renal functional and hormonal decompensation and in the elevation of RI. There was a coupling between the magnitude of morphological changes and RI increases. A set of the findings emphasizes the undoubtedly important role of renal ultrasound study that makes it possible to judge the state of the vascular bed and to suggest renal structural problems in congenital hydronephrosis.

The paper presents the results of morphological and immunohistochemical studies of the kidneys and ureters obtained at surgery in 25 children aged from 2 days to 3 years who had congenital obstructive uropathies. The morphological study revealed varying renal tissue dysplasia concurrent with dysplasia of the prepelvic or distal ureter. Immunohistochemically, the kidneys showed a pronounced expression of TGFbeta1, TGF(1R1 and R2 in the nephrocytes of non-differentiated and collecting tubules, in the tubular lumen, vascular walls, in the interstitial tissue and cell interstitial infiltrates, on the hypoplastic and disoriented ureteral smooth muscle fibers. The use of the ILIAS-method to determine urinary TGF levels before and after surgery indicated a direct correlation of these indices with the degree of renal tissue dysplasia.

Biopsies of veins of 20 boys with varicocele were studied morphologically. Three types of veins were observed. The first type was represented by veins with a wide lumen and the wall consisting of three layers of smooth muscle cells. The second morphological variant--veins of varying diameter of irregular shape and thin, frequently sclerosed wall. The third type were veins of a small diameter with well developed muscular layer. Immunohistochemical studies detected disturbances of vascular wall components suggesting the development of varicocele as a complex of congenital pathology with secondary alteration and destabilisation of compensatory processes.

Two cases of Gaucher's disease of the juvenile type are described, in a boy of 1 year and 7 months and in a girl of 8 years. The juvenile type of Gaucher's disease is documented by detection in the internal organs of typical Gaucher's cells formed mainly of histiocytes and macrophages, PAS-positive and weakly positive with Sudan III, and by the absence of the brain gangliocytes impairments. The peculiarity of these cases consists in the combination of glucocerebroside with congenital developmental defects of the urinary system: in one case with bilateral megaureter and hydronephrosis and in the second one with developmental defect of the renal arteries and bilateral cystic renal dysplasia.

Analysis of section material was carried out covering the period 1943-1975. It was established that proportion of septic diseases in the general structure of children's mortality in the recent years was stabilized at rather high levels (1973-1975--17.1%). Fatal outcomes were most often among infants at the early period of life in whom sepsis developed against the background of premorbid factors including prematurity, malformations, pathologic labour, mother's diseases, etc. The course of sepsis was particularly severe in infants with congenital immunodeficient states. The main pathogene of sepsis at present is staphylococcus. During the period under study, correlation and morphology of various forms of sepsis varied depending upon the pathogene and therapy applied. Since 1968 and up to new the most common form of sepsis has been that morphologically identical to sepsis observed in 1943-1947, i.e. before the wide application of antibiotics.

The main changes occuring in the theory of thesaurismoses are presented briefly. Main groups of thesaurismoses characterized by disorders of one or another kind of metabolism are dealt with. Most frequently occuring forms of thesaurismoses are described and by their examples it is shown how new nosological entities are distinguished by the nature of metabolic disorders, by the defect of the enzyme, and by the degree of severity of the enzymatic block. The main criteria for morphological diagnosis of thesaurismoses are presented including the presence of accumulation cell, their localization in different organs, histochemical analysis of accumulation products and electron microscopic findings. Biochemical examinations are considered to be essential for diagnosis.

Kidney biopsies were examined in 73 children with some forms of obstructive uropathies: vesico-renal reflux and obstructive megaurether. In 59 patients (80%), chronic pyelonephritis was detected. The predominant morphological form of chronic pyelonephritis in patients with obstructive uropathies is a mixed form with a combination of tubulo-stromal-vascular changes. In most patients chronic pyelonephritis was extensive up to the development of nephrosclerosis, in 22% it developed against the background of renal dysplasia. The nature and intensity of pyelonephritis was shown to depend on the severity of congenital pathology of the urinary tracts and the age of the patients.

The main pathology leading to mortinatality has been determined. It is shown that in 57.8% of cases mortinatality is associated with placental insufficiency, the reason for which can be found out by the pathoanatomical study of the afterbirth. It is proposed to differentiate afterbirth disorders of implantation, placentation, circulation and inflammatory changes. Fifty per cent of neonates are shown to die from the respiratory failure associated with the formation of atelectasis, hyaline membranes and edema with pulmonary hemorrhages. Next in frequency are cephalic ventricle extravasations. The main pathogenetic factor of both neonatal pathologies is prematurity with functional and morphological tissue immaturity.

A septic form of yersiniasis caused by Yersinia enterocolitica in a 3 1/2 year-old boy with congenital renal pathology, Fanconi's nephronophthisis, is described. The morphological features of the observation included fibrinoid necroses of the blood vessels of internal organs of the type of periarteritis nodosa.