Effect of growth hormone therapy on adult height of children with turner syndrome

Background/Purpose: Short stature is a common manifestation of Turner syndrome. The purpose of this study was to evaluate the effect of growth hormone (GH) therapy alone on the adult height of children with Turner syndrome. Methods: From 1987 to 2006, 21 Turner syndrome patients who had been treated with GH for > 2 years and had reached adult height were enrolled in the study. The dosage of GH was 0.33 mg/kg/week. Estrogen replacement therapy was prescribed at the age of 15.6 +/- 0.9 years, if indicated. The patients had been followed-up until they reached their adult height. During the same period, 28 Turner syndrome patients who were not treated with growth-promoting agents were enrolled for comparison. Mann-Whitney U test and Wilcoxon signed rank test were used for comparison. Results: Twenty-one patients in the study group started GH therapy at the age of 11.5 +/- 1.8 years. The duration of GH therapy was 4.0 +/- 1.5 years. The growth rate before treatment was 3.8 +/- 0.7 cm/year, which increased to 7.1 +/- 1.4, 5.4 +/- 1.4 and 4.7 +/- 0.9 cm/year during the first 3 years of GH therapy, respectively. Patients who received GH reached an adult height of 150.0 +/- 5.1 cm, which was significantly higher than the 144.7 +/- 5.9 cm of the control group (p < 0.05). The adult height of the study group was 6.3 +/- 3.3 cm taller than their projected adult height upon enrolment. No major adverse events were detected during GH therapy. Conclusion: GH alone is safe and effective for the promotion of growth in children with Turner syndrome in Taiwan.     

Clinical characteristics and management of acute suppurative thyroiditis in children.

BACKGROUND: Acute suppurative thyroiditis is an uncommon disease in children. This paper describes the clinical characteristics and management of children with acute suppurative thyroiditis treated during a 15-year period at National Taiwan University Hospital. PATIENTS AND METHODS: From 1985 to 2000, acute suppurative thyroiditis was diagnosed in 11 previously healthy children (6 boys, 5 girls) at the Department of Pediatrics. Their mean age at diagnosis was 6.4 +/- 4.4 years. Leukocyte count, acute-phase reactants, thyroid function, and thyroid autoantibodies were assessed. Samples were taken by thyroid needle aspiration for cytology study and pus culture. Underlying pyriform sinus fistula (PSF) was demonstrated by barium esophagogram. RESULTS: Leukocytosis was noted in six cases (55%) and acute-phase reactants were elevated in eight cases (73%). Neither thyroid autoantibodies nor thyroid dysfunction was detected in any of the patients. Barium esophagogram detected PSF in eight of 10 patients examined. Five (45%) patients had recurrent suppurative thyroiditis before surgery. Cytology and pus cultures were available for 10 patients. Polymorphonuclear cells were the main findings in the smear from thyroid aspirates. Twenty-two organisms were isolated from six patients (60%). Streptococcus species (45%) and anaerobic organisms (41%) were the most common pathogens isolated. Mixed infection was detected in five of six children who had a causative microorganism identified. The microorganisms were all sensitive to amoxicillin-clavulanate. CONCLUSION: PSF plays a role in the pathogenesis of acute suppurative thyroiditis in children. Streptococcus species are the most common pathogens in acute suppurative thyroiditis. Our results suggest that amoxicillin-clavulanate is the drug of choice for the treatment of this disease.     

Outcome of infants with hypoplastic left heart and Turner syndromes

OBJECTIVE: To report the obstetric and neonatal outcomes of ten infants with hypoplastic left heart syndrome in association with Turner syndrome. METHODS: The Pediatric Cardiovascular Surgery database at the University of Michigan was searched from 1990 to 1997, and obstetric and neonatal records of neonates with hypoplastic left heart syndrome and Turner syndrome were reviewed. RESULTS: There were 406 cases of hypoplastic left heart syndrome admitted during 8 years, of which ten (2.5%) also had Turner syndrome. Nine infants were delivered at term and one at 36 weeks. The mean (+/- standard deviation [SD]) gestational age at delivery was 38 +/- 1.2 weeks, and mean (+/-SD) birth weight was 2991 +/- 438 g. Delivery was vaginal in all cases, and no infant had an Apgar score at 5 minutes less than 7. Karyotype was 45, X in seven cases, and 45, X mosaic in three. Most infants had dysmorphic features at birth. All ten infants had first-stage reconstruction surgery for hypoplastic left heart syndrome. Only two survived and underwent second-stage palliation; both are alive currently, although with significant medical problems. CONCLUSION: For infants with hypoplastic left heart and Turner syndromes, regular obstetric management appears appropriate. Although staged reconstruction surgery has improved survival for neonates with isolated hypoplastic left heart syndrome, for those with Turner syndrome, survival appears markedly reduced.     

Prevalence of perinuclear antineutrophil cytoplasmic antibody in patients with Graves' disease treated with propylthiouracil or methimazole in Taiwan.

BACKGROUND AND PURPOSE: Antineutrophil cytoplasmic antibody (ANCA)-mediated vasculitis can be induced by propylthiouracil (PTU) and methimazole (MMI) for the treatment of Graves' disease. This study investigated the prevalence of ANCA positivity and its clinical correlates in Taiwanese patients with Graves' disease treated with PTU or MMI. METHODS: Eighty nine patients with Graves' disease who were currently being treated with PTU (n = 47) or MMI (n = 42) were included in the study. Sera were screened for ANCA by indirect immunofluorescence. The antigenic specificity of myeloperoxidase or proteinase-3 was measured by enzyme-linked immunosorbent assay. Thyroid autoantibodies against microsomal antibodies (AMA) and thyroglobulin antibodies were detected by indirect passive hemagglutination assays, and thyroid autoantibodies against thyrotropin receptor were detected by a radioreceptor assay. The correlations among ANCAs, clinical manifestations, gender, duration of treatment, and thyroid autoantibodies were analyzed by logistic regression. RESULTS: 20.2% of patients with Graves' disease receiving PTU and MMI were seropositive for ANCA; all of them were perinuclear-ANCA (p-ANCA)-positive. The frequency of p-ANCA-positive status in the PTU treatment group was significantly higher than in the MMI treatment group (31.9% vs 7.1%; p = 0.01). The mean duration of PTU treatment was 25 +/- 16 months, and was 30 +/- 21 months for the MMI treatment. In the PTU treatment group, the average duration of treatment in p-ANCA-positive patients was significantly longer than in p-ANCA negative patients (32.9 +/- 16.3 vs 19.6 +/- 12.1 months, p = 0.04). In addition, the prevalence of AMA positivity was significantly lower in p-ANCA-positive patients than in p-ANCA negative patients (53.3% vs 90.6%; p = 0.01). CONCLUSIONS: Only p-ANCA positivity was found in long-term treatment with PTU and MMI in Graves' disease. A higher frequency of p-ANCA positivity was found in the PTU treatment group than in the MMI treatment group. However, the presence of AMA was less frequent in p-ANCA-positive patients compared to p-ANCA-negative patients treated with PTU.     

Premenarchal and postmenarchal girls with insulin-dependent diabetes mellitus: ovarian and other organ-specific autoantibodies, menstrual cycle

STUDY OBJECTIVE: To estimate various organ-specific autoantibodies and detect other endocrine autoimmune disorders and menstrual cycle characteristics in girls with Type 1 insulin dependent diabetes mellitus (IDDM). DESIGN: Prospective cohort study from 1993 to 1998, duration 4.5 years. SETTING: Diabetes & Endocrine Clinic of the University Hospital, Motol, Prague. PATIENTS: 53 IDDM girls (group A--43 postmenarchal, group B--10 premenarchal), 15.5 +/- 2.5 (8-19) years old, 6.2 +/- 4.3 years after IDDM onset. MAIN OUTCOME MEASURES: Ovarian autoantibodies directed to ooplasm, zona pellucida, membrana granulosa, theca folliculi interna, and lutein cells, insulin autoantibodies, thyroid peroxidase and thyroglobulin autoantibodies. Menstrual cycle character, endocrine glands disturbance. Diabetes control, body mass index, duration of IDDM. RESULTS: Ovarian autoantibodies in at least one of the followed structures were found in 67.9% of the IDDM girls. In the control group of 21 healthy girls of corresponding age, the positive findings in lutein cells were found in only 4.8% of the girls (P < 0.01 versus IDDM girls). The lutein cells commonly associated with theca folliculi interna cells were the most frequent immunopositive structures in diabetic girls (P < 0.05 versus another positive ovarian autoimmune structure). Autoantibodies directed to ovarian steroid producing cells were frequent in IDDM patients with both irregular and normal menstrual cycles. Irregular menstrual cycles were diagnosed in 27.9% of IDDM girls, polymenorrhea in half of them, and oligomenorrhea in the remainder. Diabetes control in our patients (glycosylated hemoglobin HbA1c in postmenarchal girls 10.1 +/- 2.0%) did not differ between those with regular and those with irregular menstrual cycles. Over a follow-up period one-third of the girls with oligomenorrhea and a long-term noncompliance (HbA1c 13.5%) developed secondary amenorrhea. Insulin autoantibodies were found in 67.8%, thyroid peroxidase autoantibodies in 12.5%, and thyroglobulin autoantibodies in 10.4% of the IDDM girls. Autoimmune thyroiditis was diagnosed in 5 IDDM patients (9.4%); hypothyroidism developed in 3 of them. Menstrual cycle was irregular in 4 of the 5 girls with autoimmune thyroiditis (polymenorrhea in 1, oligomenorrhea in another 3 girls). CONCLUSIONS: An increased incidence of various circulating autoantibodies may be markedly demonstrated in IDDM girls. Their reproductive function might have an important relationship to an evidence of ovarian autoantibodies. Menstrual cycle disturbances could be linked to the poor diabetes control, to the presence of ovarian and other autoantibodies, and also to other autoimmune disease.     

High prevalence of chronic thyroiditis in patients with polycystic ovary syndrome

Objective

A higher prevalence (26.9% versus 8.3% of controls) of autoimmune thyroiditis (AIT) in polycystic ovary syndrome (PCOS) has been reported in one study to date. We aimed to evaluate the prevalence of clinical, subclinical, potential thyroid autoimmune diseases and other organ-specific autoimmunity in a group of Italian patients with PCOS.

Study design

113 consecutive patients referred to our endocrinology unit as outpatients over 18 months, and diagnosed with PCOS according to the Rotterdam criteria, were included in the study, and 100 age-matched healthy women were enrolled as controls. Each patient was evaluated for family and personal history of autoimmune and non-autoimmune diseases and tested for autoantibodies against thyroperoxidase, thyroglobulin, parietal cells, intrinsic factor, adrenal-cortex, 21-hydroxylase, steroid-producing cells, 17-alpha-hydroxylase, side-chain cleavage enzyme, islet-cells, glutamic-acid decarboxylase, nuclei and mitochondria. All patients had serum TSH, FT4 and FT3 tested and patients with thyroid autoantibodies and/or abnormal TSH levels had an ultrasound thyroid scan. An oral glucose tolerance test and measurements of serum anti-Mullerian hormone (AMH) and inhibin B levels were carried out.

Results

AIT was present in 30/113 (27%) patients compared with 8% of controls (p < 0.001). Subclinical hypothyroidism was detected in 13/30 (43%) patients with AIT; the remaining patients had normal thyroid function. The prevalence of non-thyroid autoantibodies in PCOS patients was not different from controls. AMH concentration was higher in PCOS patients compared to controls, but there was no difference between AIT and non-AIT groups.

Conclusions

The prevalence of AIT in patients with PCOS was significantly higher than in controls. No other autoimmune diseases were associated with PCOS. This observation suggests that PCOS patients should be screened for AIT.     

Renal malformations in children with Turner's syndrome.

Between 1988 and 1999, renal sonography and intravenous urography were performed to detect renal malformations in 54 patients with Turner's syndrome (TS). The mean age of these patients at diagnosis of TS was 9.2 +/- 4.6 years. Renal malformations were detected in 21 patients by intravenous urography and there was no significant difference in the frequency of renal malformations among different karyotype groups. Horseshoe kidney was the most common renal malformation, followed by duplex kidney. Fifteen of 21 renal malformations were not detected by renal sonography. We conclude that these TS patients had a high frequency of renal malformations, and that the detection rate of horseshoe kidney and duplex kidney by renal sonography was not satisfactory. Although renal sonography alone can be used to detect more severe renal malformations that may need further management, it may underestimate the frequency of renal malformation in children with TS.     

Clinical improvement following home parenteral nutrition in pediatric patients with intestinal failure.

BACKGROUND/PURPOSE: Home parenteral nutrition (HPN) is being increasingly used to treat children with intestinal failure. This study evaluated the long-term growth, outcome and complications in Taiwanese pediatric patients with intestinal failure who were treated with HPN. METHODS: This retrospective study included 27 consecutive pediatric patients with intestinal failure who received long-term HPN between 1987 and 2002. These patients were categorized into two groups according to whether they had short bowel syndrome or a bowel motility disorder. Growth, prognosis and complications, including cholestasis, hypoglycemia, hyperglycemia and infections were compared between the two groups. RESULTS: The median age of starting HPN was significantly younger in patients with short bowel syndrome (5 months) than in patients with motility disorders (1.9 years). The median duration of HPN treatment in the overall group was 13.5 months (range, 2.1-113.1 months); weight and height increased 1.7 +/- 2.3 and 1.0 +/- 1.6 in z score, respectively. The most common complications were cholestatic liver disease (52%), hypoglycemia (15%) and hyperglycemia (33%). All patients maintained stable serum glucose levels at follow-up. Cholestatic liver disease developed after 2.3 +/- 2.0 months of total HPN in 13 patients, which subsided after 9.7 +/- 6.9 months in 11 patients, while two patients died. The mean incidence of central venous infection was 3.0 +/- 3.3 per 1000 HPN days. The most common pathogens were Staphylococcus spp. (50%) and Candida spp. (30.6%). CONCLUSION: HPN treatment can successfully provide a bridge to enteral nutrition in pediatric patients with intestinal failure. The metabolic disturbances and cholestasis are usually transient, but infection control is important throughout the period of HPN treatment.     

Islet cell and other organ-specific autoantibodies in Chinese children with insulin-dependent diabetes mellitus

Islet cell autoantibodies (ICA) and other organ-specific autoantibodies were detected in 27 Chinese children with insulin-dependent diabetes mellitus (IDDM). The frequency of ICA within the first year of diagnosis was 67%, and it declined as the duration of the disease increased. The frequencies of thyroid microsomal autoantibodies and gastric parietal cell autoantibodies were 26% and 11%, respectively. None of the patients had adrenal autoantibodies. These results are comparable to those reported in Caucasian patients. Similar frequencies of islet cell autoantibodies and other organ-specific autoantibodies in children with IDDM between Chinese and Caucasians suggest that the autoimmune process may play an important role in the pathogenesis of Chinese children with IDDM.     

Intellectual outcome of patients with congenital hypothyroidism detected by neonatal screening.

BACKGROUND AND PURPOSE: Mental retardation is a major sequela of delayed treatment for congenital hypothyroidism; congenital hypothyroidism can be treated early if detected with neonatal screening. We evaluated the intellectual outcomes of 62 patients with congenital hypothyroidism detected by neonatal screening at a major teaching hospital in northern Taiwan. The effects of thyroid pathology, age at the initiation of treatment, socioeconomic status, and severity of hypothyroidism on intellectual outcome were also analyzed. METHODS: All patients had euthyroid status at the time of intelligence testing. The Chinese Fourth Revision of the Binet-Simon Scales was used to evaluate the patients' intelligence between the ages of 3 and 6 years. RESULTS: The mean intelligence quotient (IQ) score was 102 +/- 18. Only four of the 62 patients were mentally retarded. Patients with lower initial serum thyroxine concentrations (T4; < 2 micrograms/dL) at the time of diagnosis of congenital hypothyroidism had significantly lower IQs (95 +/- 19, n = 26) than those with higher initial T4 concentrations (106 +/- 16, n = 36; p < 0.05). Patients with fewer than three ossification centers had lower IQs (91 +/- 20, n = 12) than those with three or more (104 +/- 17, n = 36; p < 0.05). Significantly lower IQs were also found in patients with a smaller femoral epiphysis area (< 0.1 cm2) (92 +/- 20, n = 15) than in those with larger epiphyses (106 +/- 15, n = 21; p < 0.05). The type of pathology (ectopia, athyrosis, dyshormonogenesis), age at the start of treatment (before or after 30 days of age), and socioeconomic status did not significantly affect the intellectual outcome. CONCLUSIONS: Our results indicate that intellectual outcome in Taiwanese patients with congenital hypothyroidism has been improved by neonatal screening and that the severity of hypothyroidism at diagnosis is the most important prognostic factor affecting intellectual outcome in these patients.     

甲状腺自身抗体与复发性流产

摘要：甲状腺自身抗体是一种以自身甲状腺组织作为靶抗原的自身抗体,主要包括抗甲状腺过氧化物酶抗体、甲状腺球蛋白抗体及促甲状腺激素受体抗体,三者均是反映自身免疫性甲状腺疾病的特异指标。甲状腺自身抗体的存在可能与复发性流产有相关性,应对高危人群进行甲状腺自身抗体的筛查、早期诊断并及时给予治疗,可有效降低妊娠不良结局的发生。     

Neoplastic disorders of hematopoiesis in children with Down's syndrome--a single institution experience in Taiwan.

BACKGROUND AND PURPOSE: Children suffering from Down's syndrome (DS) are predisposed to the development of neoplastic disorders of the hematopoietic system and tend to display many unique characteristics. This study analyzed the clinical characteristics and treatment outcomes of 16 children with DS who developed neoplastic disorders. METHODS: All DS patients aged < 18 years of age with a diagnosis of leukemia or myelodysplastic syndrome (MDS) from 1990 to 2002 were included in this retrospective study. The clinical and laboratory characteristics of patients, including age at diagnosis, gender, initial hemogram, cytogenetic findings, immunophenotype, treatment regimen and outcomes were analyzed. RESULTS: Sixteen patients met the inclusion criteria. Among them, 9 patients (56%) had acute myeloid leukemia (AML), mainly of the megakaryoblastic subtype. All 8 AML patients who had analyzable metaphase cells revealed clonal chromosomal abnormalities in addition to trisomy 21. Three of these patients developed MDS prior to the onset of AML. Of the 5 patients who underwent chemotherapy, 3 remained in remission with a survival time of 29, 59, and 109 months, and the remaining 2 died as a consequence of chemotherapy toxicity. Among the 6 patients (38%) who developed transient myeloproliferative disorder, 2 were lost to follow-up, 2 died from DS-associated congenital heart abnormalities and 2 survived without any AML changes. The remaining 1 patient (6%) who developed ALL was still in his first remission although this patient suffered profound chemotherapy complications during treatment. CONCLUSIONS: This study found that AML is the most common hematologic neoplasm in Taiwanese children with DS, especially megakaryoblastic leukemia. This finding is comparable to the reported results from related studies in different countries. Long-term remission of AML in DS patients can be achieved with appropriate treatment.     

The prevalence of autoantibodies in pregnant and nonpregnant women

The frequency of autoantibodies during pregnancy was studied. Sera from 136 women (84 pregnant and 52 nonpregnant) were tested for antibodies directed against nuclear antigens, smooth muscle antigens, gastric parietal cells, mitochondria, and striated muscle by indirect immunofluorescent microscopy. Rheumatoid factor was measured by a latex agglutination test, and thyroid microsomal and thyroglobulin antibodies were measured by a hemagglutination assay. Immunofluorescence assays were considered positive if antibodies were detected at a serum dilution of 1:20. All positive samples were retested at increasing dilutions until immunofluorescence or agglutination could not be detected. The control population consisted of 52 normal, healthy, ovulatory, and nonpregnant women. The study population consisted of 84 pregnant women, none of whom had a known autoimmune disorder. The mean ages of the two groups were 32 and 26 years, respectively. The frequency of autoantibody detection was not significantly different in comparing the study (46.2) and control groups (40.5). The results did not support the hypothesis that pregnancy has an effect on the induction or suppression of autoantibody production. Previous studies that suggested differences may have been influenced by the lack of a control group.     

Physiological changes of adrenal androgens in childhood.

BACKGROUND AND PURPOSE: To investigate the change in serum dehydroepiandrosterone sulfate (DHEAS) and androstenedione concentrations during childhood and to clarify the relationship between adrenarche and gonadarche. METHODS: A cross-sectional study of 577 healthy children (297 boys and 280 girls), with ages ranging from 5 days to 12 years was conducted. After complete physical examination and recording of any sign of puberty, blood samples were drawn for the determination of serum DHEAS and androstenedione concentrations. For comparison, blood samples were also drawn from 39 normal adults, 23 men and 16 women. RESULTS: In both genders, the DHEAS and androstenedione levels at birth were 3.0 +/- 1.3 mumol/L and 4.1 +/- 2.3 nmol/L, respectively. They fell rapidly within 6 months, and were maintained at a low level until 6 years of age. After the age of 6, the levels of both adrenal androgens were significantly increased (p < 0.05). Adrenarche occurred at the age of 7.7 +/- 1.1 years in boys and at the age of 7.7 +/- 1.0 years in girls. Gonadarche occurred at the age of 11.2 +/- 0.8 years in boys (n = 33) and at the age of 10.3 +/- 0.8 years in girls (n = 46). The onset of adrenarche occurred about 2 to 3 years earlier than the onset of puberty (p < 0.001). CONCLUSIONS: Serum levels of adrenal androgens change dramatically during childhood. Serum concentration of DHEAS is a good marker for adrenal androgens production because gonadal androgens may interfere with serum concentration of androstenedione. For Taiwanese children, the onset of adrenarche occurred between the ages of 6 and 8 years, which was 2 to 3 years earlier than the onset of puberty.     

Pharmacokinetic study of recombinant human factor IX in previously treated patients with hemophilia B in Taiwan.

BACKGROUND/PURPOSE: The pharmacokinetic (PK) study of recombinant human factor IX (rFIX) has been done in patients with hemophilia B in areas other than Taiwan. However, wide patient-related variability in recovery is noted and there is no PK study of rFIX in Taiwanese patients with hemophilia B. The purpose of this study is to evaluate the PKs of the rFIX in Taiwanese patients with hemophilia B. METHODS: A PK study of rFIX was performed in 10 previously treated Taiwanese patients with hemophilia B. Nine of them had severe hemophilia B and the other one had moderately severe hemophilia B. The mean age of our patients was 24.7 +/- 8.6 years (mean +/- SD) with a range of 15.5-47.0 years. RESULTS: The infusion of 75 IU/kg of rFIX in our patients resulted in a mean FIX activity increase of 1.08 +/- 0.27 IU/dL per IU/kg with a range of 0.3-1.49 IU/dL per IU/kg, and a mean in vivo recovery of 44.0 +/- 9.5% with a range of 31.4-60.4%. The mean elimination half-life was 24.4 +/- 6.4 hours with a range of 14.1-35.3 hours. The mean values of area under the curve, total body clearance, volume of distribution at steady state were 1363 +/- 166 IU x hour/dL, 4.84 +/- 1.03 mL/hour/kg, and 144.3 +/- 41.8 mL/kg, respectively. In addition, rFIX was well tolerated in our patients. Only two patients experienced mild treatment-related adverse events, including fever and feeling sleepy, respectively. Both of them resolved spontaneously without any sequences, and there was no thrombosis formation or inhibitor development in the following 6 months after rFIX infusion. CONCLUSION: The results of PK study of rFIX in our previously treated Taiwanese patients with hemophilia B are comparable to the reported results from other related studies conducted in different geographical areas.     

Usefulness of drug eluting stent in percutaneous coronary intervention--a single center experience in Taiwan.

BACKGROUND/PURPOSE: Drug eluting stents (DES) have been shown to reduce in-stent restenosis rate and target vessel revascularization in large clinical trials. However, the safety and efficacy of DES use in the Taiwanese population has not been reported. We designed this trial to analyze the clinical results in patients using DES in a single tertiary center. METHODS: We retrospectively analyzed the clinical data of all patients treated at National Taiwan University Hospital, Taipei, Taiwan, with sirolimus- or paclitaxel-eluting stents between September 2003 and January 2005. RESULTS: A total of 585 patients (466 men, 119 women; mean age, 64.5 +/- 11.2 years) were enrolled. Meanwhile, 205 sirolimus- and 717 paclitaxel-eluting stents were implanted, with a mean of 1.6 stents per patient. Half (50.2%) of the stents were placed in the left anterior descending artery. Among the enrolled patients, 41.8% had diabetes mellitus, 25% had a diagnosis of acute coronary syndrome, and 10.7% was treated with primary percutaneous coronary intervention for ST-segment elevation myocardial infarction. Overall 8-month target vessel revascularization, major adverse cardiac event rate, and cardiac death rate were 8.8%, 9.7% and 2.5%, respectively. There was no difference in clinical events between sirolimus- and paclitaxel-eluting stents. The overall subacute stent thrombosis rate was 1.36%, significantly lower than that in patients who presented with acute coronary syndrome (4%). CONCLUSION: The use of DES in the Taiwanese population yielded comparable results as those in large clinical trials. Subacute stent thrombosis rate was higher in acute coronary syndrome. The safety of DES in these situations should be further clarified.     

Bone mineral density in women receiving thyroxine suppressive therapy for differentiated thyroid carcinoma.

BACKGROUND AND PURPOSE: Most patients with well-differentiated thyroid carcinoma have an excellent prognosis and are likely to live long enough to be subjected to osteoporosis. The purpose of this study was to investigate the consequences of treatment with a supraphysiological dose of levothyroxine (l-T4) on bone mineral density (BMD) in Taiwanese women with differentiated thyroid cancer. METHODS: A total of 69 (44 premenopausal, 25 postmenopausal) Taiwanese women with differentiated thyroid cancer were included in this retrospective study. These patients were free of disease recurrence after initial near-total thyroidectomy and I-131 radioablation, and had undergone regular l-T4 suppressive therapy for more than 3 years (mean, 7.3 +/- 3.0 years; range, 3 to 15 years). The degree of thyroid-stimulating hormone (TSH) suppression was determined based on the mean TSH score for each patient which was determined by analysis of all available follow-up TSH data, where 1 = undetectable TSH (< 0.2 mIU/mL); 2 = subnormal TSH (0.2 to 0.39 mIU/mL); 3 = normal TSH (0.4 to 4.0 mIU/mL); and 4 = elevated TSH (> 4.0 mIU/mL). The patients were divided into a full TSH suppression group with a mean TSH score in the range 1.0 to 1.99, and a partial TSH suppression group with a mean TSH score in the range 2.0 to 2.99. BMD was measured by dual-energy X-ray absorptiometry at the lumbar spine, femoral neck, Ward's triangle and total hip. Comparisons between subgroups of patients and controls were performed by unpaired t test. Correlation between BMD and other clinical variables was assessed by Pearson's correlation analysis. RESULTS: Postmenopausal patients (aged 57.7 +/- 6.9 years) had significantly higher serum calcium levels and decreased BMD at all sites of the spine and hip as compared with premenopausal patients (aged 38.6 +/- 6.7 years) with similar BMI and duration of TSH suppression. Comparison of BMD between postmenopausal patients and BMI- and age-matched controls revealed that the patient group had decreased BMD at all sites of measurement, although this difference was not significant. This phenomenon was not observed in the premenopausal patients. Furthermore, when BMD was compared between patients categorized as having full and partial suppression of TSH, only patients with full suppression in the postmenopausal group showed a tendency to lower BMD. There was a strong correlation of BMD with age, BMI and serum calcium level. However, no correlation was found between BMD and degree of TSH suppression or duration of l-T4 suppression therapy. CONCLUSION: Women with differentiated thyroid cancer who had long-term (mean, 7.3 +/- 3.0 years) l-T4 therapy and suppressed TSH levels had no evidence of lower BMD. However, patients with full suppression in the postmenopausal group showed a tendency towards lower BMD. Therefore, careful monitoring of BMD in postmenopausal women during suppression therapy is mandatory.     

Late-onset and rare far-advanced pulmonary involvement in patients with sarcoidosis in Taiwan.

BACKGROUND: Sarcoidosis is still considered a rare multisystem disorder in Taiwan, and data on the disease course and outcome are limited. We analyzed the clinical manifestations, disease course and complications in Taiwanese patients with sarcoidosis. METHODS: A retrospective cohort design was used. Fifty-six patients with sarcoidosis diagnosed between 1985 and 2004 were included. Their clinical features, laboratory findings at initial presentation, disease course, and complications were analyzed. RESULTS: Forty-three patients (76.8%) were female. The mean age at symptom onset was 47 years. The most common clinical symptoms were pulmonary (82.1%), cutaneous (23.2%), ophthalmic (19.6%), and articular (17.8%). Only two patients presented with L?fgren's syndrome. There was a seasonal variation in disease onset, with higher incidence in winter and early spring. No advanced pulmonary involvement was noted. Elevated levels of serum angiotensin converting enzyme (sACE) were found in 72.5% (29/40) of patients with active sarcoidosis, and significantly higher levels of sACE were found in patients with lung involvement (27.98+/-1.71 IU/L vs. 18.2+/-2.76 IU/L; p<0.01). In 50% (20/40) of patients, sACE levels declined significantly in parallel with clinical remission (24.75+/-1.53 IU/L vs. 16.33+/-1.21 IU/L; p<0.05). Spontaneous complete remission was found in 20.7% of patients, whereas 39.6% of patients with multiple extrapulmonary involvement responded poorly to intensive corticosteroids plus various immunosuppressants. CONCLUSION: In this series, the mean age of disease onset was in middle age (mean, 47 years old), there was a low incidence of L?fgren's syndrome (3.6%), and no patients had advanced pulmonary syndrome. The results of this study also suggest that sACE might be a marker of pulmonary involvement that is also useful in monitoring disease activity.     

Thyroid autoimmunity and infertility

We aimed to study the prevalence of thyroid autoimmunity in infertile women; to assess whether thyroid autoantibodies were associated with non-organ-specific autoantibodies; and to investigate the influence of this dysfunction on the couples' chances of pregnancy. We assayed serum levels of thyroid stimulating hormone (TSH) ,free thyroxine ,and microsomal and thyroglobulin autoantibodies in 149 infertile women. In patients with serum TSH levels in the hypothyroid or hyperthyroid range and/or with thyroid autoantibodies ,we performed thyroid ultrasound examinations and assayed some non-organ-specific autoantibodies. We compared the duration of infertility in infertile patients with normal thyroid (control group) ,with thyroid abnormalities ,and with thyroid autoantibodies in euthyroidism. Thirty infertile patients (20.1%) had thyroid abnormalities. The prevalence of thyroid autoantibodies was 17.4%. In infertile patients with thyroid autoantibodies ,we found a poor association with non-organ-specific autoantibodies. Only the women with thyroid abnormalities and ovulatory dysfunction had a mean duration of infertility significantly longer than that of the control group. When the data were analyzed for euthyroid women with thyroid autoantibodies ,we found no significant variation in the duration of infertility. Although we found a high prevalence of thyroid autoantibodies in infertile patients ,the presence of these autoantibodies per se did not reduce the chance of pregnancy.     

Familial hypopituitarism associated with mosaic form of Turner syndrome.

We present herein an unusual coincidence of familial hypopituitarism associated with a mosaic form of Turner syndrome in two adult sisters (51 and 43 years old). Both patients had hypopituitarism diagnosed in childhood. They have never been administered growth hormone, and remained short in stature. They were not given long-term estrogen-progestin treatment, despite lack of menstruation. Early in childhood both received thyroid hormone substitution. Pituitary imaging revealed pituitary hypoplasia with partial empty sella in one sister, and pituitary hypoplasia in the other. Very recently, during endocrinological evaluation, they were diagnosed with a mosaic form of Turner syndrome, additionally to their hypopituitarism. In this paper, we place special emphasis on the results of hormonal analyses and discuss the differential diagnosis.