Pheochromocytomas or Paragangliomas are neroendocrine tumors elaborating catecholamines. They may not present always in a classic manner and a high degree of suspicion is important in diagnosing them early. They can grow large enough to impinge on the great vessels and yet symptoms may not indicate the severity until late. A young African 32 year old Male presented with occasional headaches, moderate weight loss, abdominal pain and hypertension for three years. On investigation for hypertension a large paraganglioma was discovered wrapped around the Aorta and vena cava. He was operated successfully after preoperative preparation and was discharged normotensive.
Young hypertensive should always be worked up fully including checking for abdominal masses. The classic paroxysm was absent in this case. After surgical removal the catecholamines elaborated by the tumor also take time to be washed out of the circulation thus the blood pressure will need careful monitoring. Conventional alpha blockage first and beta blockage later was the algorithm followed but choice of agent was also discussed with nesthethesiologist. Though classically taught phenoxy benzamine is used, in this case it was not used and a combination of Prazosin and Propranolol
Hypertension; paraganglioma; pheochromocytoma
The forum for injection techniques, India recommendation, the first ever in the country on insulin injcetion techniques, have covered the science and the art of insulin injection technique in an exhaustive manner. However, a few gaps were identified in the document, which are addressed in the current addendum. This article focuses on insulin injection technique in special clinical situations, including geriatric people, women in pregnancy and those with dermatological or surgical disease who live with diabetes. The addendum also covers salient features of administration of insulin using the insulin pump.
Dermatology; geriatric diabetes; insulin pump; pregnancy; surgical disease
The importance of diabetes education in the management of patients is very crucial. One-on-one education, didactic group lectures are traditional methods employed in Diabetes Education. In our environment, group interaction using the Diabetes Conversation Map tools may prove to be effective especially during regular meetings of the Diabetes Association.
Conversation map; diabetes; education; Nigeria
To determine the usefulness of CD4 count in predicting adrenocortical insufficiency (AI) in persons with HIV infection.
Experimental study involving people with HIV infection and healthy people.
The participants were recruited from the Lagos University Teaching Hospital. Forty-three newly diagnosed, treatment naive persons with HIV (23 males and 20 females) and 70 (35 males and 35 females) HIV negative subjects completed the study.
One microgram Synacthen® was given intravenously to stimulate the adrenal glands.
Main Outcome Measures:
Blood was collected for cortisol at 0 and 30 min after the injection of adrenocorticotropic hormone (ACTH) and CD4 count.
Mean basal cortisol was 154.9 ± 27.2 nmol/L and 239.9 ± 31.6 nmol/L (P < 0.001); the 30-min post ACTH test, cortisol level was 354.8 ± 19.9 nmol/L and 870.9 ± 163.5 nmol/L (P < 0.001); the increment was 100.0 ± 17.2 nmol/L and 588.8 ± 143.4 nmol/L (P < 0.001) in HIV and healthy subject group; respectively. Using the diagnostic criteria for diagnosis of AI in this study, fifteen (34.8%) persons with HIV had AI. There was no significant correlation between basal cortisol levels and CD4 count in patients with HIV infection (r = -0.2, P = 0.198). There was no significant correlation between stimulated cortisol level and CD4 count in patients with HIV infection (r = -0.09, P = 0.516).
CD4 count does not predict the presence or absence of AI. ACTH stimulation of the adrenal gland remains the acceptable standard.
Adrenocorticotropic hormone; CD4 count; cortisol; human immunodeficiency virus
Diabetes mellitus is a complex metabolic disorder whose detrimental effects on various organ systems, including the nervous system are well known.
This study was conducted to determine the changes in the brainstem auditory evoked potentials (BAEP) in patients with type 2 diabetes mellitus.
Materials and Methods:
In this case-control study, 116 females with type 2 diabetes and 100 age matched, healthy female volunteers were selected. The brainstem auditory evoked potentials (BAEP) were recorded with RMS EMG EP Marc-II Channel machine. The measures included latencies of waves I, II, III, IV, V and Interpeak latencies (IPL) I-III, III-V and I-V separately for both ears. Data was analysed statistically with SPSS software v13.0.
It was found that IPL I-III was significantly delayed (P = 0.028) only in the right ear, while the latency of wave V and IPL I-V showed a significant delay bilaterally (P values for right ear being 0.021 and 0.0381 respectively while those for left ear being 0.028 and 0.016 respectively), in diabetic females. However, no significant difference (P > 0.05) was found between diabetic and control subjects as regards to the latencies of waves I, II, III, IV and IPL III-V bilaterally and IPL I-III unilaterally in the left ear. Also, none of the BAEP latencies were significantly correlated with either the duration of disease or with fasting blood glucose levels in diabetics.
Therefore, it could be concluded that diabetes patients have an early involvement of central auditory pathway, which can be detected quite accurately with the help of auditory evoked potential studies.
Auditory evoked potentials; brainstem dysfunction; diabetes mellitus; interpeak latency; sensorineural hearing loss
Soluble CD40 ligand (sCD40L) is known to be elevated in different clinical situations including hypercholesterolemia, acute coronary syndromes, and type 2 diabetes mellitus (T2DM), Data about the relationship between type 1 diabetes mellitus (T1DM) and sCD40L is limited. In addition, the potential role ofsCD40Lin the pathogenesis of vascular complications in children and adolescents with T1DM is to be clarified. Hence, the study aimed at assessment of sCD40L levels in children and adolescents with T1DM and correlation of these levels with glycemic control and microalbuminuria.
Settings and Design:
Cross-sectional controlled study.
Materials and Methods:
The study was performed in the Pediatric Endocrinology and Diabetes Unit, Assuit University Children Hospital, Assiut, Egypt. It included 70 children and adolescents with T1DM (mean age 14. 76 ± 2.21 years). Cases were further subdivided into 43 cases with normoalbuminuria and 27 cases with microalbuminuria according to presence or absence or microalbuminuria in fresh urine samples. Twentyfive healthy subjects, age- and sex-matched were included as control group (mean age = 13.62 ± 2.11 years). Studied cases were subjected to medical history, clinical examination, and laboratory assessment of fasting blood glucose (FBG), lipid profile, glycosylated hemoglobin (HbA1c), and sCD40L were performed.
Mean HbA1c and sCD40L were significantly higher in diabetic children (n = 70) compared to control (n = 25) (P < 0.001 for each). Mean HbA1c and sCD40L levels were significantly higher in microalbuminuric cases (n = 27) compared to normoalbuminuric cases (n = 43) (P < 0.05 and <0.01, respectively). We also observed a significant positive correlation between sCD40L levels and the age, diabetes duration, HbA1c, and urinary albumin creatinine ratio.
The high serum sCD40L levels in children and adolescents with T1DM particularly in those with microalbminuria and its positive correlation with diabetes duration, urinary albumin excretion, and glycemic control may reflect the role of sCD40L in diabetic vasculopathy in the pediatric age group. Moreover, measurement of serum sCD40L levels in poorly controlled patients would help to identify those at high risk of developing nephropathy.
Glycemic control; microalbuminuria; sCD40L; type 1 diabetes mellitus
Current treatment guidelines support the role of lifestyle modification, in terms of increasing the quantity and quality of physical activity to achieve target glycemia in patients with type 2 diabetes mellitus.
To assess the effect of structured exercise training and unstructured physical activity interventions on glycemic control.
Materials and Methods:
This was a randomized six-month exercise intervention study conducted with previously inactive 279 patients of type 2 diabetes mellitus. Before randomization, all enrolled T2DM participants (n: 300; 30 to 60 year old, having diabetes for more than a year with HbA1c levels of 6.5% or higher) entered a one-month run-in phase to reduce dropout and maintain adherence.
A recommendation to increase physical activity was beneficial (0.14% HbA1c reduction; P = 0.12), but was not bringing significantly declines in HbA1c, whereas, structured exercise training is associated with a significant HbA1c decline of 0.59%. (P = 0.030). In a subgroup analysis limited to participants with a baseline HbA1c value > 7%, both the unstructured (0. 48%; P = 0.04) and structured exercise training (0.77%; P < 0.01) groups experienced significant decline in HbA1c Vs the control, whereas among participants with baseline hemoglobin A1c values less than 7%, significant reduction occurred only in the structured exercise training group. Changes in blood pressure; total cholesterol, HDL-cholesterol (high-density lipoprotein), LDL-cholesterol (low-density lipoprotein) and the atherogenic index factors did not statistically significantly differ within (baseline to follow-up) and among groups.
Supervised structured training was more efficacious than unstructured activity in achieving declines in HbA1c. Although both structured and unstructured training provide benefits, only the former was associated with significant reductions in HbA1c levels. Therefore, T2DM patients should be stimulated to participate in specifically designed exercise intervention programs.
Hemoglobin A1c; physical activity; structured exercise; type 2 diabetes mellitus
The prevalence of children obesity is rising alarmingly in both developed and developing countries. Developing effective exercise programs is a strategy for decreasing this prevalence and limiting obesity-associated long-term comorbidities.
To determine whether a 16-week training program; in addition to the school physical education and without dietary intervention; could have beneficial effects on body composition and aerobic capacity of obese children.
Materials and Methods:
Twenty-eight obese children (16 boys, 12 girls; aged 12-14 years) were enrolled and were divided into either the exercise group (EG, n = 14) or the control group (CG, n = 14). EG participated in a 16-week aerobic exercises (four 60-min sessions per week at 70-85% of HRmax (maximum heart rate)), in addition to the school physical education. Fat-Free Mass (FFM) and Fat Mass (FM) were assessed with bioelectrical impedance equipment. To assess aerobic capacity, maximal metabolic equivalent of task (METmax) and maximal workload (Wmax) were estimated with an electronically braked cycle ergometer (type Ergoline 500®).
At baseline, there were no differences between the two groups. After the training program, only the EG showed significant reduction in BMI (body mass index) and waist circumference compared with the baseline values (P < 0.001). Exercise training significantly decreased FM only in the EG. A significant increase in FFM was seen in both groups; more marked in the EG. There was a significant increase in METmax (P < 0.05) and Wmax (P = 0.02) in the EG, and no significant changes in these parameters were seen in the CG. HRmax significantly decreased only in the EG (P < 0.05).
This training program has beneficial effects on body composition and aerobic capacity parameters in obese children. Our intervention has the advantage of providing a sustainable and reproducible school and community approach for the management of children obesity.
Aerobic capacity; body composition; children obesity; physical exercise
Thalassemia major patients with repeated blood transfusion have high prevalence of endocrinopathies due to iron overload.
Materials and Methods:
We examined the adrenocortical function in 23 thalassemic patients (10 children and 13 young adults) aged 8-26 years. Serum cortisol and dehydroepiandrosterone sulfate (DHEA-S) concentrations were determined in each subject before blood transfusion both in basal condition and after low dose (LD) (1 μg), followed by standard dose (SD) (250 μg, respectively) with synthetic corticotrophin beta 1-24 ACTH (Synacthen, Ciba). Normal controls were a group of 13 age- and sex-matched normal subjects.
Using a peak total cortisol cutoff level of 550 nmol/L and increments of 200 μg above basal cortisol, adrenal insufficiency (AI) was demonstrated in 8 patients (34.7%) after the LD ACTH and in 2 patients (8.7%) after SD cosyntropin (ACTH) test, but none of the controls. Using a peak total cortisol cutoff level of 420 nmol/L and increments of 200 μg above basal cortisol, AI was demonstrated in 5 patients (21.7%) after the LD ACTH and in 2 patients after SD ACTH test (8.7%), but none of controls. All patients with biochemical AI were asymptomatic with normal serum sodium and potassium concentrations and had no history suggestive of adrenal pathology. The peak cortisol concentrations in thalassemic patients with impaired adrenal function both after 1 μg and 250 μg cosyntropin (294 ± 51 nmol/L and 307 ± 58.6) were significantly lower than those with patients with normal (454 ± 79.7 nmol/L and 546.1 ± 92.2 nmol/L, respectively) and controls (460.2 ± 133.4 nmol/L and 554.3 ± 165.8 nmol/L, respectively). Adolescents and young adults, but not children with thalassaemia, had significantly lower peak cortisol concentration after SD ACTH versus controls. Peak cortisol response to LD ACTH was correlated significantly with peak cortisol response to SD in all patients (r = 0.83, P < 0.0001). In adolescents and young adults with thalassemia, DHEA-S levels before and after LD ACTH stimulation were significantly lower and the cortisol/DHEA-S ratios were significantly higher than the controls.
The use of LD ACTH test diagnoses more adrenal abnormalities versus SD ACTH in thalassemic patients. The relatively high prevalence of AI in thalassemic adolescents and young adults necessitates that these patients have to be investigated for AI before major surgery and those with impaired cortisol secretion should receive stress doses of corticosteroids during the stressful event.
Cortisol; dehydroepiandrosterone sulfate; low dose adrenocorticotropic hormone test; standard dose adrenocorticotropic hormone test; thalassemia
This paper describes the clinical diagnosis of Proteus syndrome (PS) in children referred for evaluation of asymmetric disproportionate overgrowth.
Materials and Methods:
Retrospective, descriptive, cross-sectional study conducted from January 1998 to December 2010.
During the study period, 2011 new patients were evaluated. Thirteen (0.65%) patients presented features suggestive of PS. These patients were formally evaluated based on the revised diagnostic criteria proposed by Biesecker. The mean age was 6.92 ± 5.1 years. Ten patients (76.9%) were females. All subjects had asymmetric disproportionate overgrowth. Other dysmorphic features were as follows: macrodactily (84.6%); linear epidermal nevus (41.6%); hemangioma (30.7%); and lipoma (23%). Six patients fulfilled the diagnostic criteria for PS.
The diagnostic rate of only 46.1% of patients with PS confirms the diagnostic difficulties and the need for continuous monitoring and periodic review of these patients since the clinical manifestations of this syndrome become more evident with aging. Molecular tests may help the differential diagnosis of Proteus syndrome when they became commercially available.
Asymmetric overgrowth; diagnostic criteria; proteus syndrome
Background and Objectives:
Growth parameters are important indicators of a child's overall health, and they are influenced by factors like blood glucose control in diabetic children. Data on growth parameters of Indian diabetic children is scarce. This retrospective, cross-sectional, case control study was conducted at diabetes clinic for children at a tertiary care center at Pune, to study growth parameters of diabetic children in comparison with age-gender matched healthy controls and evaluate effect of different insulin regimes and age at diagnosis of diabetes on growth.
Materials and Methods:
One twenty five diabetic children (boys: 50) and age gender matched healthy controls were enrolled. All subjects underwent anthropometric measurements (standing height and weight). Mean height (HAZ), weight (WAZ) and body mass index (BAZ) for age Z scores were calculated. Diabetes control was evaluated by measuring glycosylated hemoglobin (HbA1C). Statistical analysis was done by SPSS version 12.
Mean age of diabetic children and age gender matched controls was 9.7 ± 4.4 years. Diabetic children were shorter (128.3 ± 24.3 cm vs. 133.6 ± 24.7 cm) and lighter (29.2 kg ± 15.3 vs. 31.3 ± 15.4 kg). HAZ (−1.1 ± 1.2 vs. −0.2 ± 0.8) and WAZ (−1.2 ± 1.3 vs. −0.7 ± 1.3) were significantly lower in diabetic children (P < 0.05). Children on both insulin regimes (intensive and conventional) were shorter than controls (HAZ-intensive −1.0 ± 1.0, conventional −1.3 ± 1.3, control −0.2 ± 0.8, P < 0.05). HAZ of children who were diagnosed at <3 years of age was the least (−1.6 ± 1) amongst all diabetic children while those diagnosed after puberty (>14 years) were comparable to healthy controls.
Growth was compromised in diabetic children in comparison to controls. Children diagnosed at younger age need more attention to optimize growth.
Age at diagnosis; growth; India; insulin regimens; type 1 diabetes
Low testosterone levels are associated with an atherogenic lipid profile and may contribute to the pathogenesis of atherosclerosis.
Our study aimed to investigate the relationship between serum total testosterone (TT) levels and lipid profile in angiographically confirmed coronary artery disease (CAD) in men.
Settings and Design:
This is a case-control hospital-based study at Teaching Hospital, Karapitiya, Galle, Sri Lanka.
Materials and Methods:
Two hundred and six men, 103 with angiographically proven CAD and 103 healthy men as a control group were studied. The serum levels of TT and lipids were assessed.
Data were analyzed using Minitab software (version 15 for Windows).
The mean concentrations of lipid parameters of patients and controls were as follows: Serum total cholesterol (TCh), 5.9 ± 2.8 vs. 5.2 ± 1.6 mmol/l (P = 0.022), low-density lipoprotein cholesterol (LDL-Ch), 3.9 ± 1.2 vs. 3.1 ± 0.5 mmol/l (P = 0.001), high-density lipoprotein cholesterol (HDL-Ch), 1.1 ± 0.5 vs. 1.4 ± 0.6 mmol/l (P = 0.001), and TGs, 2.0 ± 1.0 vs. 1.5 ± 0.8 mmol/l (P = 0.001); lipid levels were significantly different between the two groups. The mean levels of TT in the patients and controls were 11.4 ± 2.7 vs. 18.1 ± 7.2 nmol/l (P = 0.001), significantly different. Among CAD patients, a significant positive association was found between testosterone and HDL-Ch (r = 0.623, P = 0.001), whereas a negative association was found with LDL-Ch (r = -0.579, P = 0.001).
Low levels of TT in men with CAD that appear together with an atherogenic lipid milieu may be involved in the pathogenesis of CAD. The observed association between testosterone and HDL-Ch suggests a protective effect of the hormone.
Coronary artery disease; lipid profile; testosterone
Almost 15% of India's urban adult populace now lives with type 2 diabetes. This study aimed to characterize the eating patterns, knowledge, beliefs, and determinants of food choice, and assess associations with the metabolic health among urban Asian Indians with type 2 diabetes.
Materials and Methods:
A cross-sectional study of 258 individuals (mean age 55.7 ± 10 years; body mass index 27.1 ± 4.8 kg/m2; diabetes duration 10.1 ± 6.5 years) attending two out-patient clinics in New Delhi, India. Food-related information was collected during a semi-structured interview. Clinical, anthropometric, and biochemical data were recorded.
Beliefs related to health and diabetes played a role determining food choice and dietary patterns; erroneous views were associated with the poor food choices and greater metabolic perturbations. Average consumption of fruits/vegetables was low. Intakes were positively associated with intentions to manage diabetes; inversely associated with the waist circumference and negatively correlated with one's degree of personal responsibility for food choice. Household saturated fat usage was common. High fat intakes were positively associated with the taste preference, ratings of perceived “health-value;” waist circumference, glycosylated haemoglobin percentage (HbA1c%) and lipids.
Strategies to enhance diabetes control among Asian Indians are required and should encourage fruit/vegetable intake, personal accountability, and consider individual beliefs and preferences. Greater emphasis and resources directed to regular dietary and behavioral counseling may assist.
Asian Indian; behavior; belief; diabetes; food choice
Diabetes mellitus (DM) causes pathophysiological changes at multiple organ system. With evoked potential techniques, the brain stem auditory response represents a simple procedure to detect both acoustic nerve and central nervous system pathway damage. The objective was to find the evidence of central neuropathy in diabetes patients by analyzing brainstem audiometry electric response obtained by auditory evoked potentials, quantify the characteristic of auditory brain response in long standing diabetes and to study the utility of auditory evoked potential in detecting the type, site, and nature of lesions.
A total of 25 Type-2 DM [13 (52%) males and 12 (48%) females] with duration of diabetes over 5 years and aged over 30 years. The brainstem evoked response audiometry (BERA) was performed by universal smart box manual version 2.0 at 70, 80, and 90 dB. The wave latency pattern and interpeak latencies were estimated. This was compared with 25 healthy controls (17 [68%] males and 8 [32%] females).
In Type-2 DM, BERA study revealed that wave-III representing superior olivary complex at 80 dB had wave latency of (3.99 ± 0.24) ms P < 0.001, at 90 dB (3.92 ± 0.28) ms P < 0.001 compared with control. The latency of wave III was delayed by 0.39, 0.42, and 0.42 ms at 70, 80, and 90 dB, respectively. The absolute latency of wave V representing inferior colliculus at 70 dB (6.05 ± 0.27) ms P < 0.001, at 80 dB (5.98 ± 0.27) P < 0.001, and at 90 dB (6.02 ± 0.30) ms P < 0.002 compared with control. The latency of wave-V was delayed by 0.48, 0.47, and 0.50 ms at 70, 80, and 90 dB, respectively. Interlatencies I-III at 70 dB (2.33 ± 0.22) ms P < 0.001, at 80 dB (2.39 ± 0.26) ms P < 0.001, while at 90 dB (2.47 ± 0.25) ms P < 0.001 when compared with control. Interlatencies I-V at 70 dB (4.45 ± 0.29) ms P < 0.001 at 80 dB (4.39 ± 0.34) ms P < 0.001, and at 90 dB (4.57 ± 0.31) ms P < 0.001 compared with control. Out of 25 Type-2 DM, 13 (52%) had diabetic neuropathy, of which 12 (92%) showed abnormal BERA. In nonneuropathic [12 (48%)] only 6 (50%) showed abnormal BERA.
Delay in absolute latencies and interpeak latencies by BERA demonstrates defect at level of brainstem and midbrain in long standing Type-2 diabetes subjects, which is more pronounced in those with neuropathy.
Auditory brainstem response; brainstem evoked response audiometry; type-2 diabetes mellitus
Background and Aims:
The psychological stress associated with type 1 diabetes (T1D) may be higher in children from developing world due to limited health resources. The aims of the study were to assess the quality of life (QoL), emotional well-being, behavioral, and cognitive profile of children/adolescents with T1D diagnosed at least 6 months prior.
Materials and Methods:
Forty-nine children with T1D, aged 6−18 years were assessed using DAWN Youth QoL questionnaire, WHO-5 Well-Being Index, Child Behavior Checklist (CBCL), and Malin's Intelligence Scale for Indian children (MISIC). The association of the scores was studied with age, gender, socioeconomic status (SES), frequency of hypoglycemia, HbA1c, and age of onset and duration of T1D.
The mean (standard deviation (SD)) for DAWN QoL, WHO-5, CBCL, and MISIC scores was 24.7 (16.7), 74.6 (19.4), 52.6 (8.8), and 96.0 (11.2), respectively. The significant associations noted were: Elevated HbA1c with poorer emotional well-being; higher negative impact on ‘symptoms of disease’ and ‘future prospects’ sub-areas of QoL; shorter duration of disease with more behavioral issues; lower maternal education with more ‘withdrawn/depressed’ behaviors and ‘worry about future prospects’; and lower SES with lower MISIC scores. Earlier onset (age <5 years) was associated with fewer behavioral problems and less negative impact on QoL.
Children with recent diagnosis, older age at onset, lower maternal educational level, elevated HbA1c, or belonging to lower SES were identified to have higher prevalence of various psychological and cognitive problems. In resource-limited settings, these children should be prioritized for behavioral and cognitive evaluation.
Behavior; children; cognition; quality of life; type 1 diabetes
The aim of this study is to determine the factors responsible for differences in the prevalence of diabetes mellitus (DM) in subjects of different social class in an urban South Indian population.
Materials and Methods:
Analyses were based on the cross-sectional data from the Chennai Urban Rural Epidemiology Study of 1989 individuals, aged ≥20 years. Entered in the analyses were information obtained by self-report on (1) household income; (2) family history of diabetes; (3) physical activity; (4) smoking status; (5) alcohol consumption. Biochemical, clinical and anthropometrical measurements were performed and included in the analyses. Social class was classified based on income as low (Rs. <2000) intermediate (Rs. 2000-5000`) and high (Rs. 5000-20000).
The prevalence rates of DM were 12.0%, 18.4% and 21.7% in low, intermediate and high social class, respectively (P < 0.001). A significant increase in the risk of diabetes was found with ascending social class (Intermediate class: Odds ratio [OR], 1.7 [confidence interval [CI], 1.2-2.3]; High class: OR, 2.0 [CI-1.4-2.9]). The multivariable adjusted logistic regression analysis revealed that the effect of social class on the risk of diabetes remained significant (P = 0.016) when age, family history of diabetes and blood pressure were included. However, with the inclusion of abdominal obesity in the model, the significant effect of social class disappeared (P = 0.087).
An increased prevalence of DM was found in the higher social class in this urban South Indian population, which is explained by obesity.
Asian Indians; diabetes; obesity; social class; socio-economic status; South Asians; urban
Primary hypothyroidism is one of the most frequent complications observed in-patients suffering from thalassemia. We investigated and reviewed the thyroid function in all thalassemic patients attending the Pediatric Endocrine Clinic of Hamad Medical Center, Doha, Qatar during the last 10 years of follow-up.
Patients and Methods:
A total of 48 patients with ί-thalassemia major between 5 years and 18 years of age. Thyroid dysfunction was defined as follows: Overt hypothyroidism (low Free thyroxine [FT4] and increased thyroid-stimulating hormone [TSH] levels >5 μIU/ml); subclinical hypothyroidism (normal FT4, TSH between 5 μIU/ml and 10 μIU/ml) and central (secondary) hypothyroidism (low FT4 and normal or decreased TSH).
A total of 48 patients (22 males and 26 females) completed a 12 year-period of follow-up. During this period, hypothyroidism was diagnosed in 17/48 (35%) of patients. There was no significant difference in the prevalence in males 7/22 (32%) versus females 10/26 (38%). Sixteen of the patients had hypothyroidism after the age of 10 years (94%). The prevalence of overt hypothyroidism had risen from 0% at the age of 7 years to 35% at the age of 18 years. None of the patients had high anti-thyroperoxidase antibody titers. Out of 17 patients, 13 patients with hypothyroidism had normal or low TSH level (not appropriately elevated) indicative of defective hypothalamic pituitary response to low FT4 (central hypothyroidism). Three patients (6.3%) had subclinical hypothyroidism (TSH between 5 uIU/ml and 10 uIU/ml and normal FT4). The general trend of FT4 level showed progressive decrease over the 12 years, whereas, TSH levels did not show a corresponding increase. These data suggested defective hypothalamic pituitary thyroid axis involving both TSH and FT4 sretion in patients with thalassemia major over time. There was a significant negative correlation between serum ferritin and FT4 (r = −0.39, P = 0.007), but no correlation was found between ferritin and TSH.
Worsening of thyroid function was observed in 35% of the studied thalassemic patients by the age of 18 years. The lack of proper increase of TSH in response to the low circulating levels of FT4 in 13/17 (76%) of these patients indicates a relatively high incidence of defective pituitary thyrotrophic function in these patients.
Ferritin; free thyroxine; growth; hypothyroidism; prevalence; thalassemia; thyroid stimulating hormone
Although the exact pathogenic processes involved in vitiligo are still unknown, its association with autoimmune disorders and endocrine dysfunction has been reported. One of its associations is with thyroid diseases. The purpose of this retrospective study was to determine the prevalence of thyroid function tests and thyroid autoantibody abnormalities in children diagnosed with vitiligo and compare the results with the literature. The laboratory documents of thyroid function tests (FT3, FT4, and TSH) and thyroid autoantibodies (TgAb and TPOAb) belonging to the pediatric vitiligo patients were studied retrospectively. Thyroid function tests and thyroid autoantibody abnormalities were detected in 20 (25.3%) of the pediatric vitiligo patients. Thirteen (16.4%) patients were evaluated as subclinical hypothyroidism, two (2.5%) were evaluated as hypothyroidism, and five (6.3%) were evaluated as euthyroidism. Thyroid autoantibodies were found to be positive in nine (11.3%) patients. Previously reported prevalence of thyroid disease in children with vitiligo ranged from 10.7 to 24.1%, and the prevalence of 25.3% determined in this study was compatible with the literature. Also, the high rate of subclinical hypothyroidism determined in these patients attracted attention to the probable development of overt hypothyroidism in a long term. Thus, our results suggest that thyroid function tests and thyroid autoantibodies should be analyzed in children with vitiligo.
Autoimmunity; child; thyroid function tests; vitiligo
High prevalence of vitamin D deficiency (VDD) has been reported throughout the India for all age groups. Increased awareness about VDD among treating physicians has led to increased prescriptions of vitamin D preparations. Based on our experience of varied clinical and radiological response with different vitamin D formulations, we decided to assess cholecalciferol content of commonly available vitamin D formulations.
Materials and Methods:
We measured cholecalciferol content of 14 commercial preparations (two in the form of tablets and 12 as sachet) available in Indian market. Lab analysis was carried out in Shriram Institute for Industrial Research by high-performance liquid chromatography.
Of the total 14 samples analyzed only 4 (28.57%) were found to be within the acceptable ranges from −90 to +125% as defined by Indian Pharmacopia while 5 (35.7%) had higher and 5 (35.7%) had lower than the acceptable range. The percentage variation in cholecalciferol content as observed from the printed ranged widely from −91% to +65%.
Our study shows a high degree of variability in cholecalciferol content of commercial preparations available in the Indian pharmaceutical market. This variation has many clinical implications as it may lead both, under treatment as well as vitamin D toxicity.
Cholecalciferol; commercial preparations
Several disorders of coagulation and fibrinolysis have been widely reported in patients with hyperthyroidism. Most reports have focused on only the venous thromboembolism risk, and few of them have studied specifically the association between hyperthyroidism and pulmonary embolism (PE). We report two cases of Graves’ disease complicated by PE. The first patient is a 32 year-old man, and the second patient is a 23-year-old female. PE was suspected on the basis of pulmonary hypertension in patient one, and clinical presentation in the other patient. The first patient had also right heart failure. PE was confirmed in both patients by a lung perfusion-ventilation scan test. Thrombophilia screen revealed normal findings in the first patient and an elevation in coagulation factor VIII in the second one. Both patients received heparin, followed by oral anticoagulant therapy. In addition, they were treated with radioactive iodine resulting in partial recovery from hyperthyroidismforpatient oneand clinical euthyroidism for patient two. The former died of acute heart failure secondary to a chest infection, while the later was lost to follow-up. In conclusion, hyperthyroidism is associated with increased risk of venous thromboembolism, including PE. Potential mechanisms involved in this association include endothelial dysfunction, decreased fibrinolytic activity, and increased coagulation factors levels. Thyroid evaluation is recommended in patients with unprovoked venous thromboembolic events. Conversely, the diagnosis of venous thromboembolism should be considered in patients with hyperthyroidism, particularly if additional prothrombotic risk factors are present.
Hyperthyroidism; pulmonary embolism; venous thromboembolism; factor VIII levels
Polyostotic fibrous dysplasia is a rare non-inheritable genetic disease due to mutation in GNAS gene. Here we present two adults who were accidentally detected lytic lesions in spine and after extensive evaluation for malignancies; was diagnosed on biopsy. Current concept of the disease and management is discussed.
Fibrous dysplasia; McCune albright syndrome; thyrotoxicosis
Nelson's syndrome refers to a clinical spectrum arising from progressive enlargement of pituitary adenoma and elevated adrenocorticotrophic hormone after total bilateral adrenalectomy for Cushing's disease comprising of hyperpigmentation, visual field defects which can be life threatening. We report here a 50-year male presenting with rapid onset of Nelson's syndrome with an unusual finding of bilateral oculomotor palsy mistakenly treated as ocular myasthenia.
Cushing's disease; Nelson's syndrome; oculomotor palsy
We are hereby reporting a case of a 72-year-old Indian man, who, in the absence of a detectable tumor, presented with symptomatic hypoglycemia in the postabsorptive state (3-5 h after meal). His serum levels of insulin and C-peptide were very high. He was not taking any hypoglycemic drug. Hypoglycemic episodes completely subsided after withdrawal of pentoprazole and incorporation of small frequent meals in the dietary plan. Six months after the initial presentation, the subject became free of hypoglycemic episodes. Although insulin autoimmune syndrome (IAS) is the third leading cause of spontaneous hypoglycemia in Japan, it is extremely uncommon in the Western Countries. Till 2009, more than 200 cases from Japan and as many as 58 cases outside Asia have been reported. To the best of our knowledge, this is the first case of IAS reported from India.
Autoimmune hypoglycemia; Hirata's disease; insulin autoimmune syndrome
Malignant changes in polyostotic fibrous dysplasia are very rare. Most common malignancies reported are osteosarcoma and fibrosarcoma, chondrosarcoma and malignant fibrous histiocytoma. Here, we report a previously diagnosed case of fibrous dysplasia who has developed leiomyosarcoma; diagnosis of which was delayed for about one year despite repeated fine needle aspiration and open biopsy.
Fibrous dysplasia; leiomyosarcoma; metastasis