Hemophagocytic lymphohistiocytosis and dengue infection: rare case report.

We describe a 21-year-old female patient returning from a journey to Cambodia, who developed dengue fever complicated with hemophagocytic lymphohistiocytosis. Hectic fever, rash, leukopenia, thrombocytopenia, hepatocellular dysfunction, a markedly elevated ferritin level, and a bone marrow demonstrating abundant hemophagocytosis were present. The patient recovered within 14 days. To our knowledge, this is only the second reported case of dengue virus-associated hemophagocytosis in Europe. As dengue is a rising pathogen in tropical import diseases, clinicians must be aware of its rare but serious complications.

Short and long-term effects of growth hormone treatment on bone turnover and bone mineral content in adult growth hormone-deficient males.

OBJECTIVE: In view of the fact that GH-deficient adults present with pronounced osteopaenia and can be considered at risk for osteoporotic fractures, we wanted to investigate the effects of biosynthetic GH replacement therapy (0.25 IU/kg/week) on biochemical indices of bone turnover and on bone mineral content (BMC) in a group of GH-deficient adult males. DESIGN: We performed a 6-month randomized, double-blind, placebo-controlled study, followed by 12-24 months of GH treatment in all patients. PATIENTS: Twenty adult males with GH deficiency of childhood onset were studied. MEASUREMENTS: We measured serum IGF-I, serum phosphate, biochemical indices of bone turnover (serum alkaline phosphatase activity, serum osteocalcin, serum carboxyterminal propeptide of type-I procollagen, fasting urinary hydroxyproline/creatinine and calcium/creatinine ratios) and bone mineral content, measured at the forearm and the lumbar spine by single and dual-photon absorptiometry respectively. RESULTS: After 3 and 6 months of GH administration, the serum levels of alkaline phosphatase, osteocalcin and carboxyterminal propeptide of type-I procollagen, and the fasting urinary hydroxyproline/creatinine ratio were significantly increased compared to placebo-treated patients (P < 0.01 to P < 0.001). During the open study phase, the values for these indices of bone turnover remained elevated above pretreatment levels (P < 0.01 to P < 0.001 at 12 months), a downward trend becoming apparent after about one year of GH treatment. BMC values showed an initial decline after 3 months of GH treatment (most likely due to an expansion of the remodelling space), followed by a significant and progressive increase above pretreatment values, reaching 7.8% for total BMC at the lumbar spine (L2-L4) and 9.9% for total BMC at the forearm, after 30 months of GH administration. CONCLUSIONS: The data of our study show that administration of substitutive doses of growth hormone to GH-deficient adult males activates bone turnover for a period of at least one year and suggests that this may have a beneficial effect on bone mass in these patients.

Bone mineral status in growth hormone-deficient males with isolated and multiple pituitary deficiencies of childhood onset.

In order to determine whether growth hormone (GH) deficiency of childhood onset affects the adult bone mineral status, we assessed bone mineral content (BMC) by photon absorptiometry in 30 full-grown GH-deficient men (8 with isolated GH deficiency and 22 with multiple pituitary deficiencies; 28 previously treated with GH) and in 30 male controls matched for age (within 4 yr) and height (within 10 cm). Forearm BMC was measured by single photon absorptiometry just proximally of the distal one third of the nondominant forearm (PBMC-2 in arbitrary units and PBMC/bone width (BW) after normalization for bone width) and at a more distal site, close to the carpal joint (DBMC-2 and DBMC/BW). Lumbar BMC was measured by dual photon absorptiometry and reported as total BMC for L2-L4 (LBMC in g) and after normalization for projected area (LBMD in g/cm2). The patients had a significantly lower BMC, both at the forearm (P less than 0.0001) and at the lumbar spine (P less than 0.005): 35.7 +/- 1.0 vs. 50.0 +/- 1.6 and 36.9 +/- 1.2 vs. 52.8 +/- 1.9 (mean +/- SEM) for PBMC-2 and DBMC-2 in patients and controls, respectively; 1.36 +/- 0.03 vs. 1.70 +/- 0.04 and 1.07 +/- 0.03 vs. 1.35 +/- 0.04 for PBMC/BW and DBMC/BW; 34.00 +/- 1.08 vs. 42.02 +/- 1.27 g for LBMC and 0.886 +/- 0.016 vs. 0.976 +/- 0.018 g/cm2 for LBMD. Both the patients with isolated GH deficiency and the patients with multiple pituitary deficiencies were osteopenic when compared to their respective controls (P less than 0.01 to P less than 0.0001 for the patients with multiple deficiencies; statistical significance reached for PBMC-2, DBMC-2, and DBMC/BW only, P less than 0.05, in the small group of patients with isolated GH deficiency). For the patients (n = 19) who had at least three serial measurements over a period of 6 to 28 months, no decrease in BMC was detected. Our findings indicate that men with GH deficiency of childhood onset present with a low adult bone mass, despite prior GH substitution in most of these subjects. The observations of a more pronounced bone mineral deficit at the forearm (20-30% lower mean values, depending on the type of measurements) than at the lumbar spine (9-19%) and the findings of osteopenia in both the patients with isolated GH deficiency and multiple pituitary deficiencies, support the view that GH deficiency per se is responsible for part of the observed deficit.(ABSTRACT TRUNCATED AT 400 WORDS)

Reduced forearm bone mineral content and biochemical evidence of increased bone turnover in women with euthyroid goitre treated with thyroid hormone.

We used single-photon absorptiometry to assess forearm bone mineral content (BMC/BW) (arbitrary units normalized for bone width) at a proximal site (PBMC/BW) and at a more distal site (DBMC/BW) in 60 women treated with 25-50 micrograms T3 or 50-100 micrograms T4 for euthyroid goitre, in 13 untreated goitre patients, and in 2 controls matched for age and menopausal state for each goitre patient. BMC/BW was not significantly different between untreated goitre patients and controls. In 36 premenopausal patients, treated for 5.8 +/- 5.4 years (mean +/- SD) a slight decrease in PBMC/BW of about 5% compared to controls to controls was observed (PBMC/BW 1.42 +/- 0.19 vs 1.49 +/- 0.13, P less than 0.05). In 24 postmenopausal patients, treated for 10.0 +/- 5.8 year, a 20% deficit in BMC/BW compared to controls was found (DBMC/BW 0.80 +/- 0.18 vs 1.06 +/- 0.20, P less than 0.001 and PBMC/BW 1.14 +/- 0.20 vs 1.42 +/- 0.19, P less than 0.001). Biochemical indices of bone metabolism in 43 pre and post-menopausal patients and 43 controls showed in the patients a higher serum alkaline phosphatase activity (AP) (P less than 0.01 and P less than 0.05 and serum osteocalcin (NS and P less than 0.05). AP was negatively correlated with TSH levels and, in postmenopausal patients, with DBMC/BW and PBMC/BW. Our results suggest that treatment of euthyroid women with moderate doses of thyroid hormone increases bone turnover with clear adverse effects on bone mineral status in postmenopausal patients.

Persistence of increased bone resorption and possible role of dehydroepiandrosterone as a bone metabolism determinant in osteoporotic women in late post-menopause.

Specific data on bone metabolism in the late post-menopause are relatively scarce. This study presents data on the biochemical, hormonal and bone mineral status of a selected group of 22 osteoporotic women with at least 1 vertebral fracture who had been post-menopausal for a minimum of 5 yr. Bone resorption parameters were elevated in over two-thirds of these patients. The serum levels of dehydroepiandrosterone and its sulphate were negatively correlated with age and years since menopause but positively correlated with bone mineral content, bone turnover parameters (urinary calcium/creatinine, osteocalcin), 1,25-dihydroxy-vitamin D levels and the 1,25-dihydroxy-vitamin D/25-hydroxy-vitamin D ratio. These findings indicate that careful evaluation of the bone turnover rate in the late post-menopause can still have an important bearing on therapeutic decisions. They also support the theory that adrenal androgens play a role in bone mineral metabolism in the late post-menopause.

[Stage I and II Hodgkin's disease with enlarged mediastinum in adults. Apropos of 24 cases]. / Maladie de Hodgkin stade I et II à gros médiastin de l'adulte. A propos de 24 cas.

We have reported a retrospective study on 24 cases of supra-diaphragmatic Hodgkin's disease Stage I and II, with massive mediastinal invasion, followed from January 1981 to October 1986 and treated first with chemotherapy and then supra-diaphragmatic mantle type radiotherapy up to a dose of 40 Gy in 20 sessions and over 26 days; inverted irradiation was given to the aorto-lumbar region and the spleen up to a dose of 30 Gy in 15 sessions over 19 days. Supplementary irradiation to the superior mediastinum on average 10 Gy in five sessions over five days was given in two cases and five Gy in three sessions over three days in four cases. After initial chemotherapy there appeared to be a complete remission in 29% (7 out of 24), there was a partial remission in 71% (17 out of 24), of which one gave a 25% response, two a 50% response and 14 gave a response of greater than 80%. After radiotherapy the remission rate appeared complete in 96% (23 out of 24). The overall survival was 90% (19 out of 21) with a mean follow up of 45 months (range 8-78 months) with a complete remission level of 85.5% (18 out of 21). For the 13 cases followed for five years the overall survival level and the level of survival in complete remission was 84.5% (11 out of 13) and 77% (10 out of 13) respectively. We have seen symptomatic post radiotherapy pneumonia. The association of chemo and radiotherapy in this limited series of patients has enabled us to obtain a satisfying duration of remission.(ABSTRACT TRUNCATED AT 250 WORDS)