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Biomedical subjects

H Hamm

Publications and source records attributed to H Hamm.

At least 19 recordsLinked to original sources
PubMed1995

[Life threatening angioedema caused by acquired C1 inhibitor deficiency associated with paraproteinemia and livedo racemosa].

A 61-year-old patient with life-threatening angioneurotic oedema was found to have an acquired C1-inhibitor (C1-INH) deficiency. In addition to lowered serum levels of C1-INH (both protein concentration and enzymatic activity), C2, C4 and CH50, which are characteristic for the hereditary form of angioneurotic oedema, markedly lowered C1q was found, which is typical for the acquired form. There were no antibodies against C1-INH. Repeated thorough examination disclosed no neoplasm, though the presence of neoplasm has often been reported to be associated with the acquired C1-INH deficiency. However, the patient showed persistent paraproteinaemia and paraproteinuria and developed livedo reticularis. Treatment with danazol resulted in a rise of the complement fraction levels and cessation of angioneurotic oedema. Paraproteinaemia and livedo reticularis persisted unchanged.

Angioedema↗
PubMed1995

Naproxen-induced pseudoporphyria: appearance of new skin lesions after discontinuation of treatment.

Non-steroidal antiinflammatory drugs (NSAIDs) are routinely used in the therapy of chronic inflammatory joint diseases in childhood. Recently the NSAID naproxen was recognized to induce pseudoporphyria. This rare photodermatitis is characterized by skin fragility and vesiculation, resulting in shallow scarring. We report 4 children with juvenile rheumatoid arthritis who developed naproxen-induced pseudoporphyria. All children had received naproxen for more than 5 months when pseudoporphyria occurred. A disorder of porphyrin metabolism was excluded by analysis of the urine, serum and erythrocytes. Previous reports on naproxen-induced pseudoporphyria described a rapid disappearance of blisters after discontinuation of treatment. However, in our patients, new lesions appeared for up to 5 weeks after discontinuation of the therapy and skin fragility was apparent for up to 6 months after cessation of treatment. Since naproxen is a widely used drug in the treatment of children with juvenile rheumatoid arthritis parents of fair-skinned children should be alerted to the possibility of this rare adverse effect.

Arthritis, Juvenile↗
PubMed1995

Sclerosis of the skin in the GEMSS syndrome. An overproduction of normal collagen.

BACKGROUND: We describe a recently observed set of autosomal dominant GEMSS (glaucoma, lens ectopia, microspherophakia, stiffness of the joints, and shortness) syndrome in a 47-year-old woman and her 23-year-old son. In addition, sclerosis of the skin, from which both patients suffered, is investigated in detail. OBSERVATIONS: The histologic examination of skin biopsy specimens obtained from the upper aspects of the backs of both patients revealed a markedly thickened dermis. Immunohistochemical examination of the dermal collagen bundles showed a collagen pattern similar to systemic sclerosis and normal control skin. In situ hybridization showed a markedly enhanced gene expression of transforming growth factor beta 1. CONCLUSION: The sclerotic skin changes in GEMSS syndrome are the result of an abnormally increased production of normal collagen that might be attributable to the enhanced in situ production of transforming growth factor beta 1.

Abnormalities, Multiple↗
PubMed1992

[Anti-basement membrane antibody disease of the lungs without clinical kidney involvement].

Anaemia (haemoglobin 10.7 g/dl) and small spotty infiltrates in both lungs were found in an 18-year-old man who had increasing haemoptysis over the preceding 3 weeks. Bronchoscopy revealed diffuse bilateral pulmonary haemorrhage. Further diagnostic measures provided no evidence of involvement of other organs, in particular the kidneys. The demonstration of anti-basement membrane antibodies confirmed the diagnosis of a disease within the group of immune-induced alveolar haemorrhage. The radiological signs in the lungs regressed over 3 weeks of administering prednisone, initially 100 mg daily, then 60 mg daily, and the patient was discharged. While being treated as an out-patient, with reduction of prednisone to 10 mg daily, the haemoptysis recurred so that a single dose of cyclophosphamide, 1.5 g, was added to the immunosuppressive treatment. There was no further haemoptysis and the prednisone was discontinued after having been given for 15 months. The patient has now been in complete clinical remission for 3 years and anti-basement membrane antibodies are no longer demonstrable. At no time was there any evidence of renal involvement in the sense of the classical Goodpasture's syndrome.

Adolescent↗
PubMed1992

The surfactant system of the adult lung: physiology and clinical perspectives.

Pulmonary surfactant is synthesized and secreted by alveolar type II cells and constitutes an important component of the alveolar lining fluid. It comprises a unique mixture of phospholipids and surfactant-specific proteins. More than 30 years after its first biochemical characterization, knowledge of the composition and functions of the surfactant complex has grown considerably. Its classically known role is to decrease surface tension in alveolar air spaces to a degree that facilitates adequate ventilation of the peripheral lung. More recently, other important surfactant functions have come into view. Probably most notable among these, surfactant has been demonstrated to enhance local pulmonary defense mechanisms and to modulate immune responses in the alveolar milieu. These findings have prompted interest in the role and the possible alterations of the surfactant system in a variety of lung diseases and in environmental impacts on the lung. However, only a limited number of studies investigating surfactant changes in human lung disease have hitherto been published. Preliminary results suggest that surfactant analyses, e.g., from bronchoalveolar lavage fluids, may reveal quantitative and qualitative abnormalities of the surfactant system in human lung disorders. It is hypothesized that in the future, surfactant studies may become one of our clinical tools to evaluate the activity and severity of peripheral lung diseases. In certain disorders they may also gain diagnostic significance. Further clinical studies will be necessary to investigate the potential therapeutic benefits of surfactant substitution and the usefulness of pharmacologic manipulation of the secretory activity of alveolar type II cells in pulmonary medicine.

Adult↗
PubMed1992

Macrophages in melanocytic naevi.

Whereas the inflammatory infiltrates of malignant melanoma have been widely investigated, little is known about the infiltrates accompanying benign melanocytic naevi. Using monoclonal antibodies directed against HLA-DR antigens, the CD1 antigen, the transferrin receptor and functionally divergent macrophage subpopulations, frozen fresh material of 87 melanocytic naevi (MN), ten primary cutaneous melanomas (PCM) and ten samples of normal skin were studied. Compared with normal skin, abundant HLA-DR+ cells were found in the stroma of MN equivalent to the quantity present in PCM. In MN we found higher numbers of dermal CD1+ dendritic cells compared with PCM and normal skin. There were more macrophages that expressed the transferrin receptor or the antigens 27E10, RM3/1 and 25F9 in MN than in normal skin but fewer than in PCM. No significant differences were found between congenital MN (n = 40), common acquired MN (n = 27) and dysplastic MN (n = 20) macrophage subpopulations. Also, no correlations were evident between macrophage infiltrates and naevus location or patients' age. Our data show that potential melanoma precursors among melanocytic naevi cannot be identified by the pattern of macrophage infiltrates.

Antigens, CD↗
PubMed1992

[Cervix cancer in HPV16-associated Bowenoid papulosis].

In a 38-year-old female patient with bowenoid papulosis of the anogenital region and an extensive carcinoma in situ of the cervix uteri, HPV16 DNA was found in the biopsy specimens and HPV16/18 DNA was detected in the cervical swab. The known coincidence of both these diseases indicates the necessity for careful regular gynaecological check-ups of patients with bowenoid papulosis.

Adult↗
PubMed1992

The genetic risk for alopecia areata in first degree relatives of severely affected patients. An estimate.

Substantial evidence indicates that genetic factors may have a role in the etiology of alopecia areata (AA). Most studies, however, provide only general information on the familial incidence but fail to specify family relationships. We therefore obtained information on the incidence of AA in first degree relatives of 348 severely affected patients. In 7% one of the parents was affected. Among the siblings of the patients 3% had developed AA, while AA was present in 2% of the children. Taking into account the age of the children, their lifetime risk was calculated to approach 6%. However, a severe type of AA is to be expected only in about 2% of the children. The degree of involvement observed in the patients did not influence the frequency and type of AA present in their first degree relatives.

Adolescent↗
PubMed1992

[Elastosis perforans serpiginosa. Considerations on the pathogenesis based on a typical case].

Elastosis perforans serpiginosa (EPS) is a rare entity belonging to the group of primary perforating dermatoses. A 13-year-old male patient with Down's syndrome developed reddish hyperkeratotic papules in a serpiginous and ellipsoid configuration on the face. Histological examination revealed transepidermal elimination of thick coarse elastic fibres from the papillary dermis. The dermal infiltrate showed an immunohistological pattern consistent with an acute cell-mediated immune response. It consisted mainly of activated T-lymphocytes, with a predominance of CD4-positive cells. Considerable numbers of CD1-positive cells were also present. Inflammatory macrophages of the 27E10 phenotype were found in considerable numbers, whereas 25F9-positive resident macrophages were almost completely absent. The role of a cell-mediated immune response in the mechanism of transepithelial elimination is discussed.

Adolescent↗
PubMed1992

[Rhabdomyosarcoma: differential diagnosis of cutaneous tumors in childhood].

Primary rhabdomyosarcoma can arise in the skin, but there are few reports on this common childhood malignancy in the dermatological literature. We report on a male infant with a cutaneous tumour growing on the right nasal bridge since his 10th week of life. Clinically the tumour mimicked pilomatrixoma. Histological and immunohistological examination of the skin tumour and of subsequent lymph node metastases revealed rhabdomyosarcoma of the alveolar growth pattern. Our patient died at the age of 4 years of disseminated organ metastases.

Biomarkers, Tumor↗
PubMed1992

[Unilateral lentiginosis--a segmental neurofibromatosis without neurofibromas].

Segmental neurofibromatosis (NF) is a usually non-inherited form of NF that is characterized by unilateral neurofibromas and/or café-au-lait spots. In partial unilateral lentiginosis (PUL), apart from unilateral lentigines, café-au-lait spots of different dimensions have been described in some patients without fulfilling the diagnostic criteria for NF. We report on three patients representing the 4th to 6th cases of segmental NF without neurofibromas. The diagnosis resulted from unilateral café-au-lait spots of different sizes in all patients and axillary "freckling" and associated skeletal alterations in two patients each. As these findings are clinically and histologically comparable to reported cases of PUL and moreover fulfil the diagnostic criteria for segmental NF, our cases support the hypothesis that PUL is a segmental NF lacking neurofibromas.

Child↗
PubMed1992

Lipoproteins and apolipoproteins in human pleural effusions.

We investigated the lipoproteins and apoproteins in human serum and pleural effusions of different origin: transudates, inflammatory exudates, and malignant exudates. Transudates had a low cholesterol content of 35 +/- 12 mg/dl (mean +/- SD) because of low levels of low-density lipoprotein (LDL) cholesterol--representing 16% of serum levels--whereas inflammatory exudates (cholesterol 92 +/- 26 mg/dl) and malignant exudates (cholesterol 86 +/- 6 mg/dl) exhibited high levels of LDL, with 67% and 69% of serum levels. Apolipoprotein (apo) B level corresponded with LDL and presented with multiple split-products in sodium dodecyl sulfate-polyacrylamide gel electrophoresis in exudative effusions. LDL levels in effusions correlated with serum levels in exudates but did not correlate with those in transudates. In contrast, lipoprotein(a) appeared in all effusions from patients with detectable serum levels. The isoforms were similar as demonstrated by immunoblotting. Differences were found in the composition of the high-density lipoprotein (HDL) fraction: transudates had cholesterol-rich HDL when compared with serum. HDL particles of malignant exudates were poor in cholesterol, and isoelectric focusing demonstrated more sialized apolipoprotein E. A strongly abnormal HDL level with accumulation of cholesterol was found in a long-standing tuberculous effusion. In conclusion, cholesterol in acute effusions is bound to lipoproteins and derived from the blood. The difference in total cholesterol levels between transudates and exudates is based on the lack of LDL in transudates. Transudates show the lipoprotein characteristics of interstitial fluid. Alterations of lipoproteins occur in chronic inflammation and in malignancy with possible de novo synthesis of apolipoprotein E by tumor cells. Lipoprotein(a) accumulates independently from LDL in the pleural space, a finding that supports the view that the physiologic function of lipoprotein(a) is located in the interstitial space.

Aged↗