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Biomedical subjects

V Mahnovski

Publications and source records attributed to V Mahnovski.

At least 19 recordsLinked to original sources
PubMed1998

Mitochondrial DNA deletion with Kearns Sayre syndrome in a child with Addison disease.

UNLABELLED: Kearns Sayre syndrome (KSS) is a multisystem disorder with a confounding variety of clinical manifestations, including ocular myopathy, pigmentary retinopathy, heart block and ataxia. Endocrinopathies are common in KSS, including growth hormone deficiency, hypogonadism, diabetes mellitus and hypoparathyroidism. A variety of deletions of mitochondrial DNA (mtDNA) are found in most cases. We report on a 5-year-old boy with Addison disease in whom further investigation revealed a 4.9 kilobase mtDNA deletion and KSS. Later he developed severe lactic acidosis and expired. CONCLUSION: The degree of mutant mtDNA heteroplasmy in various tissues on autopsy did not correlate well with the clinical manifestations, although this may be due at least in part to replacement with other tissue types. Our report is the first of non-autoimmune Addison disease in KSS and patients with KSS should be evaluated for adrenal insufficiency. Early recognition of adrenal insufficiency is crucial to prevent mortality from this cause.

Acidosis, Lactic↗
PubMed1998

Gastric duplication-colonic fistula with colonic ulceration and bleeding.

An 11-month-old girl had massive rectal bleeding. A midabdominal mass was palpated. Ultrasound scan showed a large cystic mass with diffusely echogenic contents; Tc-99M radionuclide scan confirmed a duplication. Exploratory surgery and pathology examination showed a gastric duplication with formation of a fistula into the transverse colon with a colonic ulcer and hemorrhage. This combination of findings is presented as a rare cause of lower gastrointestinal hemorrhage in infancy.

Colonic Diseases↗
PubMed1997

Surgical treatment of testicular trauma: effects on fertility and testicular histology.

Unilateral testicular trauma in the postpubertal male can lead to alterations in semen analysis, but it is not clear what effect this has on fertility. To better understand how surgical treatment of testicular trauma affects both fertility and testicular histology the following study was performed. Eighty postpubertal Lewis rats were divided into eight equal groups with one group serving as a control. In the 70 remaining rats the left testicle was subjected to blunt or penetrating injury. The testicles were either left untreated, were removed, or were repaired with sutures or mesh before being returned to the scrotum. Following recovery, each male was allowed to mate to determine fertility. Fertility rates were significantly lower in all postinjury groups except the postinjury orchiectomy group. Histological analyses showed nonspecific inflammation, smaller tubules, and impaired spermatogenesis in all postinjury testicles regardless of the type of treatment. Contralateral testicles had no evidence of autoimmune injury and were essentially identical to the control group. In the postpubertal Lewis rat, unilateral testicular trauma leads to impaired fertility unless the injured testicle is removed soon after the injury. Various methods of repairing the injury did not improve fertility. In spite of the impaired fertility, the contralateral testicle appears histologically normal.

Animals↗
PubMed1996

Use of tubularized peritoneal free grafts as urethral substitutes in the rabbit.

As an alternative to bladder mucosa, free grafts of tubularized peritoneum were used as urethral substitutes in a rabbit model of hypospadias. In group 1, six mature rabbits underwent partial penile urethrectomy followed by interposition of a 2-cm-long peritoneal-lined tube graft. These animals had urethrograms performed at 3 months and were killed at 6 (n = 4) or 12 (n = 2) months. In group 2, six rabbits underwent total penile urethrectomy with placement of 3-cm-long grafts. These animals were killed 1 to 4 weeks after surgery. Clinical assessment and gross examination of the 12 rabbits showed no urinary retention, two small fistulas at the proximal anastomosis, and no strictures or diverticular. At 6 and 12 months (group 1) the urethra had healed completely and the graft edges were not visible. In group 2, 1 to 4 weeks after surgery the graft was intact and the interface between the graft and native urethra was visible. Histological studies of the grafts were compared with control peritoneum. At 1 week, a high-density single-cell layer was present. Beginning at 2 weeks, a multilayered epithelium was present, which became more organized in the older grafts. Neovascularity became visible in the subepithelial layer at 2 weeks. Acute inflammatory cells were present early and were replaced by a palisading layer of lymphocytes and plasma cells in the older grafts. Minimal fibrosis was observed. Tubularized peritoneal free grafts are a promising new urethral substitute. Graft placement is technically simple. Replacement of the peritoneum by a multilayered epithelium resembling transitional epithelium occurs early after graft placement. As much as 1 year later, minimal scarring is present.

Animals↗
PubMed1996

Fibroproliferative disorder of the antrum after an alkali ingestion.

We describe a 2 1/2-yr-old Chinese boy who ingested potassium carbonate solution and presented with gastric outlet obstruction. He underwent successful antral resection. A severe fibroproliferative process of the antral submucosa obliterating the lumen was found to be the cause of this gastric outlet obstruction. To the best of our knowledge, this is the first report describing such a fibroproliferative process of the stomach.

Burns, Chemical↗
PubMed1993

Whole organ evaluation of collagen in the developing human larynx and adjoining anatomic structures (hyoid and trachea).

The collagen composition (types I, II, and III) of the normal developing human larynx and trachea was examined by biochemical methods. Autopsy specimens of larynges with attached upper tracheal rings were obtained from 28 humans ranging in age from birth to 44 years. The specimens were randomly collected, but excluded if laryngeal disease existed. The age, sex, and cause of death were documented. Collagen is important in the growth, development, repair, regeneration, and structural and functional integrity of the laryngeal framework. A preliminary report of selected cartilaginous components of the larynx was previously published by the authors, which studied the changes in the phenotypic expression of the collagen genes in children from the newborn period to 5 years 10 months of age. The current study included all of the functioning components of the skeletal larynx and trachea. The results of biochemical examination of these tissues are reported, and the potential clinical significance of the results of the study is discussed.

Adult↗
PubMed1993

Rectal mucosal major basic protein in infants with dietary protein-induced colitis.

Dietary protein-induced colitis is a frequent cause of rectal bleeding in infants. The exact pathogenic mechanism is unknown but the disorder has been thought to be due to an allergic response. Rectal mucosal edema and eosinophilia are typically found but there are no specific markers currently available. Because eosinophil degranulation, as evidenced by the release of major basic protein, has been implicated in hypersensitivity disorders, we aimed to assess major basic protein deposition as a marker of dietary protein-induced colitis occurring in young infants. Suction rectal biopsies from five infants aged 1 to 7 months with findings consistent with dietary protein-induced colitis were compared histologically with five age matched controls who underwent rectal biopsies to rule out Hirschsprung's disease. An established indirect immunofluorescent staining method was used to identify tissue major basic protein. Comparable rectal deposition of major basic protein was found for the controls and colitic patients. Mucosal eosinophilia but not mast cell content was more prominent in the colitic patients (P < .05) than in the controls. Some of the colitic infants had elevated serum IgE levels (1 of 5), positive RAST for milk (2 of 5), and peripheral blood eosinophilia (1 of 5). Our findings do not support the concept that dietary protein-induced colitis of infancy is due solely to an immediate hypersensitivity response. The results also indicate that major basic protein is probably not a marker or likely primary mediator of this disorder.

Biopsy↗
PubMed1992

Collagen in the developing larynx. Preliminary study.

The primary purpose of this study was to determine the types of collagen in the developing human larynx that contribute to the structural framework and function of various components of this organ. The infant larynx is much more than a mere miniature of the adult "voice box." There are many age-related differences that occur in the larynx from the newborn period to the adult period of life. While collagen has been studied in numerous tissues, both normal and diseased, there have been no studies of the whole organ content, types, and/or changes of collagen in the developing human larynx that may account for many of the clinical findings. This study may at least in part explain whether collagen differences may account for the structural changes and responses that are seen in clinical practice.

Child, Preschool↗
PubMed1991

Hepatic pathology in pediatric acquired immunodeficiency syndrome.

In a retrospective study we assessed the hepatic changes in children with the acquired immunodeficiency syndrome by reviewing 12 biopsy specimens and 48 autopsy specimens from 54 children. Hepatopathology differed in biopsy and autopsy material. In biopsy specimens, chronic active hepatitis with predominantly T8 lymphocytes by tissue immunochemistry was common (five of 12 specimens). Fatty degeneration and hepatocellular necrosis were either absent, mild, or patchy. On the other hand, at autopsy, chronic active hepatitis was not observed. The most prominent changes were extensive fatty degeneration, nonspecific portal mononuclear infiltration, portal fibrosis, and confluent (ischemic) necrosis. Opportunistic infections such as Mycobacterium avium-intracellulare (MAI) were noted only at autopsy. In addition, three unusual morphologic characteristics were noted: nodular lymphoplasmacytic portal infiltrate, a pseudosarcomatous variant of Mycobacterium avium-intracellulare infection, and multinucleated giant cells (foreign both type and giant cell transformation of hepatocytes).

Acquired Immunodeficiency Syndrome↗
PubMed1988

Variable expression of Lactobacillus casei cell wall-induced coronary arteritis: an animal model of Kawasaki's disease in selected inbred mouse strains.

Mice receiving a single intraperitoneal injection of Lactobacillus casei cell wall fragments in aqueous suspension develop an asymmetric inflammatory coronary arteritis which histologically mimics the lesions seen in the coronary arteritis of children with Kawasaki's disease. A large variety of mice with genetically determined defects of the immune system were evaluated in this study to determine the influence of these defects on disease expression. Only the C3H/Hej mouse with defective macrophage function and poor production of IL-1 and TNF following stimulation with LPS failed to develop disease. This study suggests that further evaluation of macrophage function in children with Kawasaki's disease may provide important clues to its pathogenesis and treatment.

Acute Disease↗
PubMed1988

Animal models of vasculitis. Lessons we can learn to improve our understanding of Kawasaki disease.

Kawasaki disease is an inflammatory illness of unknown etiology which is often complicated by coronary arteritis. Systematic study of the pathologically similar coronary arteritis induced in mice by a single intraperitoneal injection of Lactobacillus casei cell wall fragments may provide useful clues to the pathogenesis and appropriate treatment of Kawasaki disease. Studies of mouse strains with inbred immune deficiencies suggest that the coronary artery lesions may result from macrophage-mediated injury rather than immune complexes or a disturbance of T cell or B cell function.

Animals↗
PubMed1985

Coronary arteritis in mice following the systemic injection of group B Lactobacillus casei cell walls in aqueous suspension.

We describe the induction of an asymmetric, focal, inflammatory coronary arteritis by a single intraperitoneal injection of group B Lactobacillus casei cell wall fragments in various inbred mouse strains. This coronary arteritis resembles the arteritis which is responsible for the 1-2% fatality rate among children with mucocutaneous lymph node syndrome. Coronary arteritis developed in 18 of 26 C57BL/6, 14 of 26 A/J, 7 of 15 Balb/c, and 8 of 15 C3Heb/FeJ mice injected. It also developed in 2 of 4 "nude" A/J background mice and 3 of 4 "nude" C57BL/6 mice, but in 0 of 15 C3H/HeJ mice. Lesions were evident as early as 3 days following injection. The development of arteritis was accompanied by disruption of the arterial intima and media with true aneurysm formation. Measurement of serial IgG and IgM titers indicated no relationship between the development of coronary arteritis and immunoglobulin response to L casei cell walls or the development of antibodies cross-reactive with normal myocardium. The absence of disease in only the C3H/HeJ mice, which are known to have defective macrophages, suggests that macrophages may play an essential role in the pathogenesis of coronary arteritis.

Animals↗
PubMed1982

Congenital asplenia and anomalies of the gastrointestinal tract.

Congenital asplenia has been traditionally described in association with cardiopulmonary anomalies (Ivemark syndrome). A case of congenital asplenia with duplication of the hindgut and genitourinary tract, myelomeningocele, situs inversus abdominis, and imperforate anus generated interest in review of the gastrointestinal anomalies of 36 patients who died of the disease in Childrens Hospital of Los Angeles. A significant number were found to have situs inversus, malrotation of the bowel, esophageal varices, duplication and hypoplasia of the stomach, Hirschsprung's disease, imperforate anus, and duplication of the hindgut. These patients present with symptoms referable to their gastrointestinal anomalies and may not draw attention to the concomitant cardiopulmonary anomalies or to the absence of the spleen. It becomes important, therefore, to establish the presence of the spleen in infants suffering from various anomalies of the gastrointestinal tract because of the overwhelming septicemia that is known to complicate the clinical management of these patients.

Adolescent↗
PubMed1977

Fulminant hepatitis. A presentation of Wilson's disease.

A boy, 10 years of age, was admitted to the hospital with rapid onset of hepatic failure and died within three weeks. Laboratory and pathological data were consistent with Wilson's disease. We discuss the importance of Wilson's disease in the differential diagnosis of acute liver failure.

Child↗