Familial hemophagocytic lymphohistiocytosis associated with disseminated T-cell lymphoma: A report of two siblings

Summary Two siblings with evidence of disseminated T-cell lymphoma at the time of diagnosis of familial hemophagocytic lymphohistiocytosis (FHL) are reported, an association which has not been described previously. The first child with typical clinical and laboratory features of FHL died shortly after admission, before diagnosis could be established. Retrospective analysis of autoptic tissue revealed marked hemophagocytosis as well as morphological and immunohistochemical features suggestive of disseminated T-cell lymphoma. In the second child, FHL was diagnosed in time. Subsequent histologic investigation of bone marrow biopsies displayed a focal infiltration by T-cell lymphoma. DNA hybridization studies provided evidence of a monoclonal T-cell receptor beta chain gene rearrangement. Following conventional chemotherapeutic induction for FHL, the patient received an allogeneic bone marrow transplant (BMT) from a related healthy donor. Currently, 17 months after BMT, the boy is in unmaintained remission from FHL and T-cell lymphoma. The current pathogenetic concepts for FHL and a possible relationship between T-cell lymphoma and FHL are discussed.     

Fracture of the tibial tray of the PCA knee: A case report of early failure caused by improper design

Knee prostheses with metal backing of the polyethylene tibial component are nowadays used almost universally. Eight cases of fracture of the metal tray are reported in the literature. We present a case in which the failure could be expected because of improper design of the tibial tray.     

Serum lipoproteins in acromegaly before and 6-15 months after transsphenoidal adenomectomy

OBJECTIVES Acromegaly is a rare disorder characterized by over-secretion of GH, most often because of a pituitary adenoma. The disease is associated with disturbances in lipoprotein metabolism and an increased cardiovascular mortality. The aim of the present study was to investigate whether treatment of acromegaly results in changes in serum concentrations of lipids and apolipoproteins, Including lipoprotein(a) (Lp(a)). DESIGN Fourteen patients with clinical features of acromegaly and Increased GH secretion were studied 1-10 months before and 6-15 months after transsphenoidal adenomectomy in an open study. PATIENTS Three patients had diabetes mellitus before surgery and two of these patients had normalized serum glucose levels post-operatively. Mean and baseline plasma GH levels were determined from 24-hour GH profiles. Serum samples were taken in the morning after an overnight fast. All patients were normocholesterolaemic, and four patients were hypertriglyceridaemic before treatment. RESULTS Mean plasma GH levels decreased from 34·5 ±7·4 to 2·1 ±0·4 μ/l (mean ±SEM). Serum IGF-I, insulin and free T3 levels decreased and serum SHBG concentrations Increased post-operatively. There was no effect of treatment on serum cholesterol concentrations, but serum triglyceride concentrations decreased. Serum apollpoprotein (apo) B and apoE levels were unaffected by treatment. Serum apoA-I levels increased and Lp(a) levels decreased post-operatively. CONCLUSIONS Successful treatment of acromegaly, resulting in normal mean GH values (< 5mU/l) and/or normal responsiveness to TRH, have beneficial effects on serum lipoproteins with increased serum apoA-I levels and decreased serum levels of triglycerides and Lp(a). These effects seem to be independent of improvement In glucose tolerance, since patients with diabetes mellitus before surgery and normal fasting blood glucose levels post-operatively had similar lipoprotein responses to treatment as those with normal fasting blood glucose levels before surgery.     

Extensive brain injury in a premature infant following a relatively minor maternal motor vehicle accident with airbag deployment.

Traumatic injury following a motor vehicle accident during pregnancy has an enormous potential for fetal injury and demise. With the advent of seat belts, shoulder restraints and airbags, and improved maternal survival, the most common cause of fetal loss is placental injury. However, the safety of airbag deployment during pregnancy and in particular during the latter stages, and the potential for fetal trauma remains unclear. We report a case of extensive neurological injury of a premature infant with minimal maternal trauma associated with deployment of an airbag following a minor motor vehicle accident.     

In vitro method for measuring synthesis rates in the intervertebral disc

A method for culturing intervertebral disc tissue in vitro under conditions that control changes in hydration and minimize loss of proteoglycans has been developed. Disc slices were enclosed in small-pore dialysis tubing and incubated in medium containing polyethylene glycol (PEG). By varying the PEG concentration, different external swelling pressures could be applied to the tissue. The rate of glycosaminoglycan synthesis in rabbit and dog disc was then measured using [35S]sulphate as a precursor. Synthesis rates varied with final hydration of the tissue, and the highest rates were found at hydrations close to those found in vivo. Under conditions of controlled hydration, rabbit nucleus has a higher rate of glycosaminoglycan synthesis than annulus. However, the rates measured in sagittal slices of dog disc decreased from the outer to the inner regions of the anterior annulus, reaching a minimum value in the nucleus and then increasing again in the posterior part of the annulus. The results show that in the intervertebral disc, measured rates depend on culture conditions.     

Water maze learning and hippocampal synaptic plasticity in streptozotocin-diabetic rats: effects of insulin treatment

Streptozotocin-diabetic rats express deficits in water maze learning and hippocampal synaptic plasticity. The present study examined whether these deficits could be prevented and/or reversed with insulin treatment. In addition, the water maze learning deficit in diabetic rats was further characterized. Insulin treatment was commenced at the onset of diabetes in a prevention experiment, and 10 weeks after diabetes induction in a reversal experiment. After 10 weeks of treatment, insulin-treated diabetic rats, untreated diabetic rats and non-diabetic controls were tested in a spatial version of the Morris water maze. Next, hippocampal long-term potentiation (LTP) was measured in vitro. To further characterize the effects of diabetes on water maze learning, a separate group of rats was pre-trained in a non-spatial version of the maze, prior to exposure to the spatial version. Both water maze learning and hippocampal LTP were impaired in diabetic rats. Insulin treatment commenced at the onset of diabetes prevented these impairments. In the reversal experiment, insulin treatment failed to reverse established deficits in maze learning and restored LTP only partially. Non-spatial pre-training abolished the performance deficit of diabetic rats in the spatial version of the maze. It is concluded that insulin treatment may prevent but not reverse deficits in water maze learning and LTP in streptozotocin-diabetic rats. The pre-training experiment suggests that the performance deficit of diabetic rats in the spatial version of the water maze is related to difficulties in learning the procedures of the maze rather than to impairments of spatial learning.     

Growth rate of lung metastases and S-phase fraction as determined by flow cytometry from the primary tumour in 25 patients with bone or soft-tissue sarcomas.

A significant correlation (r = -0.48) was found between the logarithm of the S-phase fraction of the primary tumour (SPF) and the logarithm of the doubling time of lung metastases (T2). The estimated median cell loss factor was 88% (range 35-99%).     

Depression in children with cancer.

The results of a standardized evaluation of the occurrence of depression in a group of 34 children suffering from cancer are presented. The children (13 male, 21 female) were admitted to the oncological unit of the Department of Pediatrics at the University of Graz, Austria, during a 14 months-course (January 1987-March 1988). We rated each child 4 times in 4 weekly intervals after admission and start of induction chemotherapy. To access the occurrence and severity of depression and to monitor progress during treatment we performed a psychiatric interview and also applied the psychiatric rating scale CDRS (Children's Depression Rating Scale) by Poznanski et al 1979 and SCMDD (Self-Report Symptom Checklist for Major Depressive Disorders) by Kashani et al 1985 derived from DSM III. 340 diagnostic ratings were performed by 3 raters; two of them were psychotherapeutically trained pediatricians (M. D., P. J. S.) and one psychologist (L. W.). A "clearcut" depression was found in 25 of the 340 ratings i.e. in 6 of the 34 children, demonstrating that the occurrence of depression in pediatric cancer patients was lower than primarily assumed.