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Biomedical subjects

J Rapola

Publications and source records attributed to J Rapola.

At least 55 records · Page 3Linked to original sources
PubMed1993

Neonatal benign sacrococcygeal teratoma may recur in adulthood and give rise to malignancy.

BACKGROUND: The capacity of neonatal sacrococcygeal teratomas (SCT) to recur is a well-recognized phenomenon. However, only a few studies have reported recurrence of the tumors beyond childhood. METHODS: A follow-up of patients for the detection of late recurrent SCT was performed in 45 patients, ages 4-43 years (mean, 21.5 years). All of the patients had been operated on in infancy for a benign SCT. RESULTS: Three adults with persistent or recurrent SCT were found. The recurrent tumors were diagnosed 21-43 years after the initial diagnosis and operative treatment. Two recurrences were histologically benign, and one was malignant. In the patient with the malignant recurrence, the coccyx was not removed primarily. The malignant recurrence was a mucinous adenocarcinoma and probably originated from a preexisting benign epithelial component of the teratoma. CONCLUSIONS: The capacity of a benign SCT to recur may be retained into adulthood. Follow-up of patients after operation for a SCT, even when the tumor is benign, should extend far beyond infancy. Abdominal radiographs may help detect late recurrent tumors with intrapelvic calcifications. When any recurrence is found, malignancy should be suspected.

Adenocarcinoma, Mucinous↗
PubMed1993

Progressive unilateral hypertrophic myopathy: a case study.

A 12-year-old girl had progressive unilateral muscle hypertrophy limited to the sole, tibialis anterior, and biceps femoris muscles. The affected muscles showed complex repetitive discharges by electromyography, necrosis and variation of fiber size upon histopathological examination, and increased metabolic activity in biochemical studies. The findings suggest a myopathic origin, but the actual cause remains unknown.

Child↗
PubMed1993

Post-transplantation nephrosis in congenital nephrotic syndrome of the Finnish type.

Congenital nephrotic syndrome of the Finnish type (CNF) is an autosomal recessively inherited disease manifesting as massive proteinuria, edema and ascites in the neonatal period. The disease is believed to be limited to the kidneys and recurrences after renal transplantation have not been reported. At our center 29 transplantations have been performed on 28 CNF patients. One to 33 months after transplantation, seven grafts (24%) of six patients have developed a steroid-resistant nephrotic syndrome. The clinical data and renal histology of these patients were analyzed in order to elucidate the cause of the proteinuria. At the onset of six of the seven episodes of nephrosis, the patient had evidence of a preceding CMV- or EBV-infection and the remaining patient had sinusitis. Upon light and electron microscopy examination, endothelial swelling of the glomerular capillaries resembling transplant glomerulopathy (TG) was seen, but unlike TG, the glomerular basement membranes were normal. The response of proteinuria to steroid or cyclophosphamide therapy was poor, with total remission in only two patients and partial remission in one patient, all treated with methylprednisolone and cyclophosphamide immediately after the diagnosis. Four grafts have been lost. Our data show that CNF patients have an increased tendency for post-transplantation nephrosis.

Biopsy↗
PubMed1993

Altered serum lipid profile after systemic infection in children: risk factor for CHD?

Systemic infections may thicken the intima of coronary arteries and modify the serum lipid profile. Infants and children are particularly susceptible to such intimal thickening, the signs of which are more pronounced in infants who have evidence of infection at death. The topography of the thickenings, their greater size in males, and in families with a history of coronary heart disease favours the idea that the thickenings are pre-atherosclerotic. Infections modify the serum lipid pattern: serum high density lipoprotein (HDL) cholesterol concentration decreases and stays low during convalescence. Thus repeated infections might prove to be a risk factor for atherosclerosis via their effect on HDL concentration.

Adolescent↗
PubMed1993

Nonspecific diskitis in children. A nonmicrobial disease?

Eighteen patients were treated for nonspecific diskitis (diskitis without microbial cause) at the University Children's Hospital in Helsinki during 1970-1990. The mean age at admission was three years three months. The characteristic findings were restriction of spinal mobility and an elevated erythrocyte sedimentation rate (ESR). Radiographic narrowing of the affected disk space was seen four to five weeks after the initial symptoms. An operative biopsy was made in 16 patients, the histologic diagnosis being chronic or subacute nonspecific inflammation in ten, other nonspecific changes in two, and normal tissue in five. Treatment was either bed rest and antimicrobial medication, or bed rest alone. After a follow-up period of five months to nine years, all patients were clinically normal. All but two had radiographic signs of healed disk space defects. Nonspecific diskitis in children is an entity with unknown cause. The clinical picture is typical, but diagnostic difficulties may be encountered during the first weeks when radiographic signs are absent. The disease is self-limiting in most cases, and only minor radiographic changes remain after healing. The results after routine operative biopsy raise the question of a possible nonmicrobial cause.

Biopsy↗
PubMed1992

Cyclosporin reduces renal prostanoid excretion in type 1 diabetic patients.

Prostacyclin and thromboxane A2 are important regulators of kidney blood flow. To examine whether changes in their metabolism could be involved in the nephrotoxicity of cyclosporin, we determined urinary excretion of 6-keto PGF1a and dinor-6-keto PGF1a (prostacyclin metabolites) and dinor-TxB2 (thromboxane metabolite) in five newly diagnosed type 1 diabetic patients during and after stopping cyclosporin therapy. In the resting state, cyclosporin had no effect on prostanoid excretion. In response to exercise, urinary excretion of 6-keto PGF1a was reduced by 50% (P less than 0.02), dinor-6-keto PGF1a by 15% (P less than 0.05) and dinor-TxB2 by 45% (P less than 0.02), while albumin excretion increased 4.5-fold (P less than 0.05) during cyclosporin therapy. Simultaneously, there was a rise in serum creatinine concentration, and renal biopsy specimens obtained from three patients showed periglomerular and interstitial fibrosis and tubular atrophy. After the discontinuation of cyclosporin therapy, serum creatinine concentrations returned to normal, histological changes improved and there was an associated rise in urinary prostanoid excretion. These data suggest that a reduction in renal prostanoid synthesis by cyclosporin may diminish renal blood flow and function, and lead to histological changes in the kidney.

6-Ketoprostaglandin F1 alpha↗
PubMed1992

The glycosaminoglycan content of renal basement membranes in the congenital nephrotic syndrome of the Finnish type.

A decrease in the concentration of heparan sulphate proteoglycan (HSPG) in the glomerular basement membrane (GBM) is supposed to cause the increased GBM permeability in the congenital nephrotic syndrome (CNS). Therefore, we analysed the glycosaminoglycan (GAG) content and composition of the GBM and tubular basement membrane (TBM) from 3 patients with CNS of the Finnish type (FCNS) and 16 control infants. The GAG content, determined by spectrophotometric assay after papain digestion, was not significantly different in FCNS patients compared with controls. In addition, the GAG composition was comparable in the two groups, with heparan sulphate (HS) constituting at least 75% of the total GAG content. The urinary GAG content (expressed as mg GAG/mmol creatinine) was age dependent, but similar in both groups. Indirect immunofluorescence studies on kidney tissue from normal human infants, using monoclonal or polyclonal antibodies against the core protein of human GBM HSPG, showed linear staining of almost all renal basement membranes. A monoclonal antibody directed against the HS chain of HSPG showed strong GBM and a weak TBM staining. Kidney tissue from three patients with FCNS displayed no discernible differences in the distribution or quality of staining with the same antibodies. These biochemical and immunohistochemical results are in contrast to the decrease in anionic sites (by polyethyleneimine staining) and the replacement of GBM HS by chondroitin sulphate, observed by others in CNS of the diffuse mesangial sclerosis type.

Basement Membrane↗
PubMed1992

Nonvacuolar myopathy in a large family with both late adult onset distal myopathy and severe proximal muscular dystrophy.

Late adult onset distal myopathies usually show vacuolar degeneration as a characteristic feature in muscle pathology. In this study vacuolar degeneration was not present in 12 patients with late adult onset distal myopathy. All patients were members of a large kindred, with 26 patients showing this new form of distal leg myopathy. Additionally, a severely disabling proximal muscular dystrophy appeared in eight other members of the large consanguineous kindred. Muscle biopsies were obtained from clinically affected muscles, and from clinically unaffected muscles in patients with distal myopathy. For comparison specimens from various muscles of patients with severe proximal dystrophy were also studied. Histopathological changes correlating to muscular dystrophy were extensive in all muscles studied in patients with proximal dystrophy, and in tibial anterior muscles in patients with distal myopathy. Mild myopathic changes, mainly increased internal nuclei in muscle fibers, were detected in clinically unaffected muscles in the distal myopathy. The spectrum of findings is compatible with the hypothesis of previous clinical and genetic studies, indicating that the severe proximal dystrophy could be a homozygous manifestation of the dominantly inherited gene of the distal tibial muscle dystrophy.

Adult↗
PubMed1992

Reversible mitochondrial myopathy with cytochrome c oxidase deficiency.

Two siblings, a boy and a girl born in a nonconsanguineous marriage, presented with a similar clinical course. Sucking and breathing difficulties appeared within a few weeks of birth. Clinical examination revealed profound muscular hypotonia, hepatomegaly, increased serum creatine kinase activities, and lactic acidosis. Both infants were treated with gavage feeding, the boy also needing ventilatory support. Clinically they improved gradually. Now, the boy aged 4 years and the girl aged 28 months are free of clinical signs. Muscle biopsy specimens taken at 3 months showed, in both, ragged red fibres, abnormal mitochondria, and reduced cytochrome c oxidase (COX) staining. Biochemical analysis showed COX activity to be reduced to about 25% of the normal mean. The second biopsy specimen from the boy at 16 months was normal on morphological examination, but the girl's second specimen at 13 months still showed abnormal features. These cases are examples of the rare benign reversible COX deficiency. Early diagnosis is crucial to provide intensive treatment until spontaneous clinical improvement appears.

Biopsy↗
PubMed1992

Chronic rejection of rat renal allograft. III. Ultrastructure of vascular and glomerular changes.

The main histological features for 'chronic' rejection of rat renal allograft are ongoing perivascular and interstitial inflammation, vascular intimal hyperplasia and glomerular sclerosis. In this communication we have investigated the ultrastructural features of renal parenchyma by comparing chronically rejecting rat renal allografts immunosuppressed initially with cyclosporine to similarly immunosuppressed syngeneic transplants. Electron microscopy confirmed that the primary arterial change was smooth muscle cell proliferation in media, thickening and focal destruction of the internal elastic lamina, and influx of smooth muscle cells and their proliferation in the intima, leading to a nearly complete occlusion of the lumen. In the glomeruli, the most prominent features were mesangial matrix increase, narrowing of capillary lumen, and basement membrane thickening with a double contour appearance in capillary walls. A thin membrane, similar to lamina densa, appeared beneath the endothelium in some segments. Mesangial interposition, formation of new basement membrane and mesangial widening due to increase of basement membrane-like material was nearly always present. As none of these vascular and glomerular changes were observed in similarly immunosuppressed syngeneic transplants, we consider these ultrastructural alterations characteristic for chronic rejection in a rat renal allograft.

Animals↗
PubMed1991

Kidney biopsy findings in cyclosporine-treated patients with insulin-dependent diabetes mellitus.

Renal biopsy specimens of 40 patients with recent-onset insulin-dependent diabetes mellitus treated with cyclosporine (CSA) for 6-29 months were examined. Cyclosporine-associated chronic vascular interstitial toxicity of moderate intensity was found in 10 patients (25%). The most prominent lesions were interstitial fibrosis and tubular atrophy. Arteriolopathy was less pronounced and glomerular damage unremarkable. A significant correlation exists between the extent of tubular atrophy and CSA trough whole blood levels. These data indicate that the development of CSA-associated chronic nephropathy is dose-dependent.

Adolescent↗