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Biomedical subjects

H Carstensen

Publications and source records attributed to H Carstensen.

At least 19 recordsLinked to original sources
PubMed1992

Tumours classified as "malignant histiocytosis" in children are T-cell neoplasms.

During the last five years increasing evidence has accumulated that many tumours classified as 'histiocytic' in the past do not originate from macrophages, but from transformed (or anaplastic) large lymphoid cells. Most of these studies have focused upon adult neoplasms. Knowledge concerning the lineage of 'histiocytic' tumours in the paediatric age group is more limited. In this study we have examined the clinical, morphological and immunophenotypical features of six childhood malignancies originally diagnosed as being of histiocytic origin. Three patients showed an aggressive course with involvement of internal organs and very short survival times. Two patients were brought into remission: one is alive without active disease after seven years; the other died after seven years due to treatment-related cardiomyopathy. The remaining patient had a protracted course for two and a half years, but subsequently deteriorated and died three years after diagnosis. The histomorphological features in five cases were those of anaplastic large cell lymphomas. The remaining case consisted of pleomorphic (rather than anaplastic) large lymphoid cells. In all cases the immunophenotypical examination showed features characteristic of activated T lymphocytes. All cases were positive for Ki-1 (CD30), and three were positive for epithelial membrane antigen (EMA). Histiocyte-associated markers were positive in residual reactive macrophages, but nowhere could unequivocal positivity for macrophage-associated markers be seen in the neoplastic cells. It is concluded that most childhood malignancies in the past classified as 'histiocytic' are examples of anaplastic large cell (Ki-1) lymphomas of T-cell type and that true histiocytic malignancies are exceedingly rare in the paediatric age group.

Antigens, CD↗
PubMed1990

Immunohistochemical study of the abnormal cells in Langerhans cell histiocytosis (histiocytosis x).

The immunophenotypic properties of the abnormal cells in routine specimens from 16 cases of Langerhans cell histiocytosis (LCH) were examined. In five cases, cryostat sections were also available. The abnormal cells expressed a similar phenotype and were positive for HLA-DR, S-100 protein, peanut agglutinin (PNA), CD1a, CD4 and several macrophage-associated markers, including CD11c, CDw32 and CD68 (the latter detectable in routine sections with antibody KP1). Staining with CD14, CD35 (C3b receptor), and CD11b (C3bi receptor) was negative with the exception of one of the cases in which a proportion of the cells showed faint positivity with CD11b. Staining for pan-T-cell (CD2, CD3, CD5) and pan-B-cell (CD19, CD22) antigens was negative in all lesions. It is concluded that LCH expresses a characteristic phenotype with some heterogeneity with regard to macrophage markers and that immunohistochemical methods in cryostat sections and routine specimens form a useful supplement to other techniques for the diagnosis of this condition.

Adult↗
PubMed1990

Hepatosplenic candidiasis in children with cancer. Three cases in leukemic children and a literature review.

Three children with acute leukemia presented with prolonged fever and neutropenia after cytostatic therapy, which was followed by abdominal pain, hepatomegaly, and hepatic dysfunction with raised serum alkaline phosphatase. Abdominal CT scan and ultrasound demonstrated multiple small lesions compatible with the hepatosplenic candidiasis syndrome. Liver biopsies showed microabscesses with a granulomatous appearance, but evidence of yeasts and pseudohyphae was present in 1 case only. Cultures were negative. Treatment with amphotericin B and 5-fluorocytosine was successful in two children. At autopsy, one child had signs of active infection. We reviewed the literature on 27 children with hepatosplenic candidiasis. Abdominal symptomatology and prolonged fever, despite antibiotic therapy, in a patient with previous or present neutropenia after cytotoxic exposure, should lead to a careful evaluation, including noninvasive imaging studies, open liver biopsy, and prompt aggressive antifungal treatment, the response to which requires close follow-up.

Adolescent↗
PubMed1990

Flow cytometric DNA analysis of lesions from 18 children with langerhans cell histiocytosis (histiocytosis x).

The DNA content in 26 formalin-fixed, paraffin-embedded histologic specimens from 18 children with Langerhans cell histiocytosis (LCH) was analyzed by flow cytometry. In two cases, the propidium iodide fluorescence histograms showed small (5 and 3% of the analyzed nuclei) but significant aneuploid subpopulations with DNA indices of approximately 1.5. This was confirmed by analysis of unfixed, frozen material in one patient. Both patients had disseminated disease without organ dysfunction and were treated with prednisone. Currently, they are without signs of disease activity after 1 and 10 years. DNA histograms were normal from a patient who died from disseminated disease and from two patients with disseminated disease who experienced several relapses and various chemotherapeutical regimens. The histograms were also normal in lesions from four patients with unifocal bone involvement. Our results show that DNA aneuploidy occurs in LCH lesions in the pediatric age group. Further investigation is necessary to reveal whether DNA aneuploidy is restricted to disseminated LCH or its presence has any value in predicting the course and outcome of the disease.

Adolescent↗
PubMed1989

[Familial hemophagocytic histiocytosis in 2 siblings].

Two siblings presented with fever, hepatosplenomegaly and pancytopenia at the age of six weeks. Subsequent investigations showed hypofibrinogenaemia, hypertriglyceridaemia, cellular-mediated immunodeficiency and hepatic and splenic lymphohistiocytic infiltrates showing haemophagocytosis. These findings are consistent with the diagnosis of familial haemophagocytic histiocytosis. Splenectomy in one infant was followed by brief improvement in the haematological parameters. Both infants died by the age of five months.

Humans↗
PubMed1989

Autoimmune involvement in Cushing syndrome due to primary adrenocortical nodular dysplasia.

Cushing syndrome due to primary adrenocortical nodular dysplasia was diagnosed in two patients, aged 3 years 9 months and 9.5 years. Subsequently, adrenalectomy was performed and followed by steroid replacement. In both cases, the adrenals were normal or only slightly enlarged and showed adrenocortical nodular dysplasia histologically. Small lymphocytic infiltrates consisting of T-cells and class II MHC positive macrophages were present in adrenal specimens of both the patients. Samples of protein A sepharose purified serum immunoglobulins from both children stimulated adrenocortical DNA synthesis and cortisol production in cultured guinea-pig adrenal segments in vitro in a dose dependent fashion. Adrenal stimulating immunoglobulins were also demonstrated in serum specimens of both patients' mothers. However, none of them had overt signs of adrenal disease. Our data support the view that autoimmune mechanisms may be involved in primary adrenocortical nodular dysplasia.

Adrenal Cortex↗
PubMed1989

Kala-azar in a four-year-old child 18 months after brief exposure in Malta.

A four-year-old Danish boy developed kala-azar 18 months after a holiday in Malta. Splenectomy, with liver biopsy, was performed six months after onset of symptoms because of hypersplenism, and the diagnosis of kala-azar was only made four months later, when the histopathological specimens were reviewed. Previous bone marrow biopsies did not show Leishmania. Treatment with sodium stibogluconate was successful. The development of kala-azar after one week's stay in an endemic area stresses the importance of including this potentially fatal disease in the differential diagnosis of cases presenting with fever, splenomegaly, and pancytopenia.

Antimony Sodium Gluconate↗
PubMed1988

Early-onset neonatal group B streptococcal septicaemia in siblings.

At each of two consecutive deliveries, a woman gave birth to a baby that developed early-onset group B streptococcal (GBS) septicaemia. A low titre of serum antibodies to the type of the infecting GBS and persistence of the organism in the mother were demonstrated. This case confirms that mothers of GBS infected infants are at high risk of their future babies being similarly infected.

Adult↗
PubMed1988

VM-26 (teniposide)-induced hypersensitivity and degranulation of basophils in children.

During a 7 year period, 16 episodes of VM-26 (teniposide) hypersensitivity occurred in our Department of Pediatrics. Eight of these (50%) were observed in neuroblastoma patients, of whom a total of 22 children had been treated with VM-26. The predominant signs were facial edema, flushing, urticaria, bronchospasm, tachycardia, and hypotension. All children with hypersensitivity recovered, but four of them were critically ill. No risk factors were found. In order to elucidate the mechanism of the hypersensitivity episode further, and to identify a possible allergen, histamine release from basophil leukocytes was performed by use of a glass microfiber method. Blood samples from nine cases reacting to VM-26, eight controls (children exposed to VM-26 without any hypersensitivity reactions), and 12 healthy children without previous exposure were challenged with VM-26 alone and with its vehicle, cremaphor. In all samples, it was found that VM-26 degranulated basophils, whereas no histamine release was seen after challenge with cremaphor. The reaction was dose-dependent, and not IgE-mediated, since IgE depletion of the cells did not abolish histamine release after VM-26 challenge.

Adolescent↗
PubMed1988

Dynamic aspects in transient hyperphosphatasaemia of infancy.

A 20-month-old boy with absence epilepsy was found to have a strikingly elevated serum alkaline phosphatase (AP) at start of treatment with valproic acid. The monitoring of this therapy made it possible to follow the elimination of AP which was shown to be a first order kinetic with a half-life of 6.1 days. A concurrent adenovirus infection was diagnosed.

Adenoviridae Infections↗
PubMed1988

Group G streptococcal neonatal septicaemia: two case reports and a brief review of literature.

We present 2 cases of early onset group G streptococcal septicaemia in full term neonates, together with a review of 15 previously reported cases, including 3 late onset cases. Most likely the neonates were infected during passage through the birth canal or had been exposed in utero. In one neonate, meconium aspiration occurred, while in the other prolonged rupture of maternal membranes was a risk factor. Both responded well to treatment with benzylpenicillin and gentamicin. Among the 12 previously reported early onset cases, 5 (43%) had a fulminant course with complications such as progressive respiratory distress, shock, and disseminated intravascular coagulation.

Adult↗
PubMed1987

Prevalence and causes of microscopic haematuria in type 1 (insulin-dependent) diabetic patients with persistent proteinuria.

The prevalence and causes of microscopic haematuria were examined in all Type 1 (insulin-dependent) diabetic patients with persistent proteinuria (diabetes duration greater than or equal to 5 years) attending the outpatient clinic at Hvidöre Hospital during 1985. One hundred eighty-four patients (69F/115M) out of 1024 Type 1 patients had persistent proteinuria (18%). Microscopic haematuria was defined as greater than or equal to 3 erythrocytes per high power field in two or more sterile urine samples. Twenty-three Type 1 patients with persistent proteinuria (7F/16M, aged 35.4 +/- 13 years) had microscopic haematuria (12.5%). No significant changes were found between the group with and without microscopic haematuria: blood pressure 148/89 +/- 22/11 versus 145/91 +/- 20/11 mmHg, duration of diabetes when persistent albuminuria occurred 17 +/- 8 versus 20 +/- 10 years, serum creatinine 99 +/- 24 versus 98 +/- 31 mumol/l, simplex retinopathy 61 versus 54%, proliferative retinopathy 39 versus 42%, and no signs of retinopathy 0 versus 4%. Kidney biopsy was performed in 13 out of the 23 patients with microscopic haematuria. Diabetic glomerulosclerosis was present in all 13 patients, but 9 patients had a non-diabetic renal disease superimposed (mesangioproliferative glomerulonephritis (n = 5), membranous glomerulonephritis (n = 3) and sarcoidosis (n = 1). Microscopic haematuria is a rare finding, frequently reflecting superimposed non-diabetic glomerulopathies, in Type 1 diabetic patients with diabetic nephropathy and well preserved kidney function.

Adolescent↗
PubMed1987

The epidemiology of cryptosporidiosis and other intestinal parasitoses in children in southern Guinea-Bissau.

In order to determine the prevalence of Cryptosporidium and other intestinal parasites, a household sample survey of children under 5 years old was carried out during the late dry season in 8 rural villages in southern Guinea-Bissau, West Africa. Cryptosporidium oocysts were found in 10 of 270 stool samples (3.7%), using a safranin-methylene blue staining method. Of these 10 children (age range 5-16 months), all non-Muslims, 6 had diarrhoea, giving a prevalence of 12.5% in 48 children with diarrhoea, compared with 1.8% in children without diarrhoea (P less than 0.001). The ethnic group with the highest prevalence (9.2%) also kept most domestic animals, and was the only group to keep cattle. Giardia lamblia was found in 16 children, and the overall prevalences of other enteric parasites were: hookworm, 21.7%; Strongyloides stercoralis, 7.4%; Ascaris lumbricoides, 6.9%; Trichuris trichiura, 4.4%; Entamoeba histolytica, 1.5%; and Taenia sp., 0.5%. The prevalence of cryptosporidiosis was highest in the age group 7-12 months, while for the other parasites it was highest in the oldest children. The prevalence of hookworm was highest (c. 50%) in the southernmost villages. No significant relationship was found between hookworm infection and anaemia.

Animals↗