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Biomedical subjects

C R Abramowsky

Publications and source records attributed to C R Abramowsky.

At least 37 records · Page 2Linked to original sources
PubMed1986

Ten-year course of early-onset Weber-Christian syndrome with recurrent pneumonia: a suggestion for pathogenesis.

A surviving 10-year-old boy with infant-onset systemic Weber-Christian syndrome is reported. He has had recurrent episodes of fever, aseptic panniculitis, and pneumonia. Although corticosteroid therapy has succeeded, colchicine and non-steroidal anti-inflammatory drugs have failed to abort or prevent acute episodes. The persistent leukocytosis (even during remission), the recurrent episodes of fever with associated increase in the acute phase reactants, and the failure of the nonsteroidal anti-inflammatory drugs leads us to propose that this form of Weber-Christian syndrome reflects an inborn error in the regulation of the inflammatory response. Systematic investigation of this hypothesis could yield important information on the normal regulation of inflammation and could lead to a rational therapeutic approach to this puzzling and usually devastating illness.

Child↗
PubMed1985

Clinical and pathologic aspects of recurrent placental villitis.

In a retrospective survey, recurrent villitis was identified in ten of 59 patients in whom placental villitis had been diagnosed. The ten patients had a total of 41 pregnancies, with a reproductive loss of 60 per cent. In addition to enhanced fetal losses in all trimesters of gestation and postnatally, the incidences of fetal growth retardation and premature delivery were increased. There was no evidence of recent TORCH (toxoplasma, rubella, cytomegalovirus, herpes) infection, but all patients tested had rubella immunity. In six patients genital cultures were positive for gonorrhea and assorted microorganisms. Uterine abnormalities, including two septate uteri, one incompetent cervix, one submucosal leiomyoma, and one retroflexion, were common, and vaginal bleeding had occurred in five patients. Other factors included obesity (five patients) and clinical and laboratory evidence of autoimmunity (four of the five patients tested). In a control group of 20 patients with nonrecurrent villitis, the perinatal loss rate (37 per cent) was lower, and the incidences of positive cultures, uterine structural anomalies, obesity, and autoimmunity were also lower. Placental histologic findings included decidual plasma cell and intervillous fibrin and histiocytic infiltration, in addition to villous inflammation. These lesions, although consistent for a given patient, defined two clinically relevant groups of patients. The results of this study suggest that recurrent villitis is more frequent than previously reported, that it is associated with high perinatal mortality, and that immunologic and structural abnormalities in the host may play a role in its pathogenesis.

Adolescent↗
PubMed1985

Intestinal lymphangiectasia and thymic hypoplasia.

We have evaluated the immunological abnormalities present in a 6 year old patient with primary intestinal and generalized lymphangiectasia confirmed by intestinal, lung and lymph node biopsies. Lymphocyte loss through the gut was confirmed by the detection of lymphocytes in her stool. An increased enteric protein loss was suggested by hypoproteinaemia, peripheral oedema, and a very short half-life for i.v. immune serum globulin (3 days). Lymphocyte subpopulation analysis revealed a selective loss of T lymphocytes, with a proportionally increased loss of the OKT4 positive helper/inducer subpopulation. Functionally, there was a decrease in proliferative responses to some mitogens and to allogeneic cells, and a lack of T cell help for in vitro B lymphocyte differentiation into immunoglobulin secreting cells. Natural killer function was normal. In this patient, a concomitant thymic deficiency was documented by failure to identify thymic tissue on a thymus biopsy and by an absence or decrease of the serum thymic factor (thymulin) and thymosin alpha 1. No compensatory lymphopoiesis was detected in the bone marrow. In an attempt to increase T lymphocyte development, the patient was treated with thymosin fraction 5. Daily treatment with this preparation resulted in a transient clinical improvement which could not be sustained on a weekly thymosin treatment schedule. However, lymphocyte numbers did not increase during this treatment. The findings in this patient support the notion that T lymphocytes are needed to stimulate thymic epithelium. In situations of excessive loss of long lived T lymphocytes a secondary thymic atrophy may occur and further contribute to the development of a deficiency in cell-mediated immunity.

Child↗
PubMed1985

Childhood orbital pseudotumor.

Pseudotumor of the orbit is an uncommon entity in children. Three patients under 20 years of age with biopsy-proved orbital pseudotumor are presented. A three-month-old infant, to our knowledge the youngest patient reported with orbital pseudotumor, had a history similar to those of previously reported cases, whereas the two older patients differed from previously reported cases of childhood pseudotumor in that they lacked constitutional symptoms and orbital pain. Two of the patients were successfully treated with systemic steroids. In one patient, radiation therapy was required to reduce the mass.

Adolescent↗
PubMed1984

Phytohemagglutinin-induced diarrheal disease.

A purified plant lectin, phytohemagglutinin (PHA), or crude red kidney bean (RKB) from which it was derived, when incorporated as 1% of dietary protein into a purified casein protein diet caused weanling rats to fail to grow or lose weight in comparison to control animals pair fed an isonitrogenous, isocaloric diet. Feeding PHA was observed to cause diarrhea: fecal wet and dry weights were increased within 2 days after starting the diet. Increased fecal weight was caused by increased dry weight as well as by an increased fecal water content. On reversion to a normal casein diet, rapid amelioration of the antinutritional effects of PHA occurred with resumption of normal growth rate. Specific binding of PHA to the microvillus region of the small intestinal epithelium was demonstrated using rabbit anti-PHA and fluorescein-labeled goat anti-rabbit immunoglobin. PHA binding was observed after chronic intake in the diet or when applied to normal tissue in vitro. Loss of PHA binding to the intestine was observed to occur within 48 hr on reversion to a control casein diet. No significant morphological damage to the microvilli or the mucosal villus architecture was observed to accompany PHA adherence under these experimental conditions. Antinutritional and antiabsorptive effects of dietary PHA were associated with diarrhea. PHA adhered to the microvillus membrane of the small intestinal villus surface during the diarrheal state.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed1984

Renal lymphangioma: a cause of neonatal nephromegaly.

A newborn male presented with bilateral nephromegaly and mild hypertension. Function of the right kidney was reduced on excretory urography. Ultrasound showed bilaterally enlarged kidneys with increased echogenicity and poorly defined corticomedullary junctions. Areas of decreased medullary enhancement were seen on CT. Renal biopsy demonstrated lymphangioma, probably arising from the peripelvic renal tissues.

Humans↗
PubMed1984

Circulating immune complexes and the nephropathy of cystic fibrosis.

To explore the putative nephropathic role of Pseudomonas-associated immune complexes, the authors measured the quantity of immune complexes in sera obtained, before death, from 20 patients with cystic fibrosis, and compared these findings with the histologic features of the lesions and with immunofluorescence patterns of kidney tissue obtained at autopsy. The immune complexes were measured by solid-phase C1q (C1q immune complex) and conglutinin to detect complexes containing IgM, IgA, and IgG. Elevated levels of C1q immune complex (13 patients) suggested the possibility of renal deposition of C3 (P less than 0.005) and IgM (P less than 0.05). The only three patients with IgA tissue deposits had elevated levels of C1q immune complex with normal IgA immune complexes. No other assay findings correlated with the immunofluorescence findings. Despite the prominent C3 in tissue deposits, the histologic features were not significantly associated with the results of the immune complex assays. This study indicates that complement-activating IgM-containing complexes can be deposited in renal tissues of patients with cystic fibrosis, but their nephropathogenicity is doubtful. These observations of kidney lesions, which diminish the injurious role of immune complexes in cystic fibrosis, may be relevant to an understanding of the pathogenesis of the lung lesions, which recent studies have linked to the presence of immune complexes.

Adolescent↗
PubMed1984

Nontuberculous mycobacterial infections: comparison of the fluorescent auramine-O and Ziehl-Neelsen techniques in tissue diagnosis.

Nontuberculous (i.e., atypical) mycobacterial infections are increasing among pediatric and immunosuppressed patients, who commonly present with subcutaneous inflammatory masses or adenitis, which is often surgically excised. Since the most frequently isolated species also grow slowly in culture, early diagnosis may depend on histologic detection of a mycobacterial organism in the biopsy specimen. However, the histologic methods used for this purpose are of uncertain value in the diagnosis of these infections. Biopsy specimens from 22 patients with clinical histories highly consistent with nontuberculous mycobacteriosis in which part of the tissue was cultured were selected for study. Coded tissue blocks and control specimens were stained by the Ziehl-Neelsen (ZN) or auramine-O (A0) fluorescent technique and examined blindly for the presence of characteristic organisms. Results of these studies were compared with the culture results, and predictive values were calculated. This experience showed that the AO technique is technically simpler, allowing faster screening at lower power and showing greater sensitivity and predictive value of a negative result although less specificity than the ZN technique. The lower specificity of AO may be factitious and due to the detection in the tissue of organisms that did not grow in culture. Previous observations that nontuberculous mycobacterial infections may elicit tissue reactions that simulate cat-scratch disease, sarcoidosis, and nonspecific chronic inflammation were also confirmed.

Adult↗
PubMed1984

Spontaneous nephrotic syndrome in a genetic rat model.

Advances in our understanding of the mechanisms of proteinuria in humans have depended on a variety of animal models. Most of these have been partially satisfactory because they require pretreatment of the animal with chemicals or toxins or they depend on an aging-related glomerular protein leakiness. The strain in this study was obtained by Koletsky after selective inbreeding of the offspring from a hypertensive Kyoto-Wistar and a normotensive Sprague-Dawley rat. The affected animals appear in 25% of the litters, indicating an autosomal recessive gene, and present with a spontaneous and progressive nephrotic syndrome detected as early as 3-5 weeks and associated with obesity, hypertension, hypoalbuminemia, hypercholesterolemia, and hyperlipidemia. Preliminary morphologic and immunofluorescence studies of their kidneys show progressive glomerular segmental sclerotic lesions and prominent mesangial deposition of IgM, a picture which resembles a steroid-resistant form of idiopathic nephrotic syndrome in humans, namely, focal glomerular sclerosis.

Animals↗
PubMed1983

Sarcoma of the larynx in a newborn.

A newborn male presented with respiratory distress and laryngeal stridor at the time of birth. Laryngoscopy revealed a circumscribed mass in the right vocal cord which was diagnosed as an undifferentiated malignant neoplasm on frozen section. Further light microscopic studies, special stains and electron microscopy disclosed features consistent with a special subtype of sarcoma adopted by the Intergroup Rhabdomyosarcoma Committee. Several authors have also demonstrated similarities between this type of tumor and soft tissue Ewing sarcomas. Nonepithelial malignancies of the larynx are rare in children and are only anecdotally reported in newborns. Histopathologically, the tumors predominantly include rhabdomyosarcomas among other rarer less well documented sarcomas and lymphomas. Although modern chemotherapy and radiotherapy have improved the otherwise grim prognosis of soft tissue Ewing sarcoma, this young patient was only treated with total laryngectomy at ten days of age and is alive and well two years later.

Cytoplasm↗
PubMed1982

Adult polycystic renal disease presenting in infancy: a report emphasizing the bilateral involvement.

We describe a girl with adult polycystic renal disease that presented as a unilateral abdominal mass on an excretory urogram. The child had a 4-generation family history of this disorder. Clinical course had been unremarkable until she was 12 years old and died of a ruptured intracranial aneurysm. Despite the clinical impression of unilateral renal involvement, postmortem examination showed that the clinically normal kidney had developed early cyst formation in several areas, thus, underscoring the bilateral nature of adult polycystic disease.

Child↗
PubMed1982

The nephropathy of cystic fibrosis: a human model of chronic nephrotoxicity.

Patients with cystic fibrosis are chronically exposed to several potentially nephrotoxic factors. These include bacterial infections with their associated immune complexes and the antibiotics (aminoglycosides) used in their treatment. In addition, diabetes mellitus, liver disease, and cor pulmonale, commonly seen in these patients, may produce renal injury. To assess the extent of this injury, we performed morphologic and immunopathologic studies of the kidneys of 34 patients at autopsy. The group included 23 female and 11 male patients; their ages ranged from 4 months to 35 years and their disease was diagnosed one month to 22 years prior to death. The histological changes included glomerulomegaly, a mesangiopathic lesion, and tubulointerstitial disease frequently associated with acute and chronic tubular injury. The last was characterized by abundant tubular lysosomal proliferation and tubular atrophy suggestive of chronic amino-glycoside injury. Diagnostic diabetic lesions were not seen. Immunofluorescence studies predominantly revealed deposits of IgM or C3, or both, in glomeruli and arterioles in 18 patients. Although an anti-Pseudomonas antiserum did not show bacterial antigens in the tissues, elution studies in two specimens demonstrated antibacterial antibodies. These observations, coupled with the finding of ultrastructural glomerular deposits, suggest immune complex-mediated injury. No correlation was found between the severity or type of renal histologic lesion and patient age or duration of cystic fibrosis. Despite the occurrence of renal failure in six patients, renal involvement is currently of limited clinical concern in cystic fibrosis. Nevertheless, continued exposure to bacterial immune complexes and aminoglycosides, among other factors, can result in potentially serious renal disease.

Adolescent↗
PubMed1981

Comparative demonstration of pulmonary fat emboli by "en bloc" osmium tetroxide and oil red O methods.

The presence and localization of fat in human lung tissue was evaluated by the "en bloc" staining procedure with osmium tetroxide performed with formalin fixed tissue with subsequent paraffin sectioning and with the oil red O technique performed with frozen sections. Fifty-one autopsy cases were divided into three clinical groups: group A, consisting of 17 patients with severe skeletal trauma; group B, consisting of 14 patients with minor skeletal trauma; and group C, consisting of 20 control patients without trauma. Adjacent sections of lung were selected from all cases, stained with the "en bloc" osmium tetroxide and oil red O methods, coded, and examined under the light microscope without knowledge of the clinical grouping, Stainable fat was graded on a 1 to 4+ scale, and attention was given to histologic localization in tissue sections. The "en bloc" osmium tetroxide technique revealed greater amounts of stainable lipid in clinical groups A and B and was most effective in demonstrating lipid when present in small quantities (group B). Since the method permits the employment of paraffin sections, evaluation of fine histologic detail is an advantage that is not always obtained in frozen sections. It is concluded that the "en bloc" osmium tetroxide technique is superior to the oil red O technique in terms of visualization and histologic localization of minute amounts of lipid in lung tissue.

Adipose Tissue↗
PubMed1981

Candida-associated renal papillary necrosis.

An autopsy series of 42 patients who had visceral candidiasis was studied to determine the incidence and clinicopathologic features of Candida-associated renal papillary necrosis. Papillary necrosis was found in nine patients (21%), associated in all instances with fungal invasion of the kidney. The single most common associated condition was prematurity, present in three neonates, and antibiotic or immunosuppressive therapy was a contributing factor in most instances. The clinical significance of candidal papillary necrosis is unclear because most patients had other causes of renal failure. Antemortem diagnosis is exceptionally difficult and had not been made in any of these patients. Pyelograms have aided in the diagnosis in only one well-documented case in the literature. This study shows that the pathologic features of candidal renal papillary necrosis correlate well with the findings in experimentally induced disease, and that this lesion appears to be more common than previously suspected.

Adolescent↗
PubMed1981

Contrast enemas in cystic fibrosis: implications of appendiceal nonfilling.

In patients with abdominal pain and suspected appendicitis, contrast examinations of the colon suggest appendicitis when there is lack of filling of the appendix, and a cecal filling defect. Complete opacification of the appendiceal lumen tends to exclude this diagnosis. In cystic fibrosis, however, the incidence and importance of these findings are unknown. The colon examinations of 29 cystic fibrosis patients who had surgery or long-term follow-up were reviewed. Some degree of appendiceal filling occurred in seven patients. Five had normal appendices of laparotomy; two were discharged. The appendices of the other 22 patients failed to fill and there were 10 cecal filling defects. Appendicitis was shown by laparotomy in only four of these patients. Although appendiceal filling indicated lack of appendicitis as in other patients, lack of visualization of the appendix is not a reliable sign of appendicitis in cystic fibrosis. Histologic studies indicate that nonfilling is frequently related to mucus plugging of the lumen.

Abdomen↗