imaging (MRI) and EEG observations, and response to immunomodulation in three cases of H.E. Case ... more imaging (MRI) and EEG observations, and response to immunomodulation in three cases of H.E. Case Reports Case 1 Mr. R, a 40-year-old-man, presented with progressive myoclonus for 9 months, cognitive decline and visuospatial disorientation for 8 months and dysarthria for 3 months. One month ago, he developed generalized tonic-clonic seizure, irrelevant speech, gait ataxia and startle response. He consumed alcohol occasionally. His general physical examination and vitals were normal. He scored 11/30 on Mini Mental State Examination (MMSE) and a detailed neuropsychological assessment revealed frontal and parietal lobe impairment. He had stimulus-sensitive multifocal myoclonus, limb rigidity and gait ataxia. The routine hemogram and serum biochemistry were normal. Chest X-ray and ultrasonography (USG) (abdomen) were normal. MRI (brain) revealed diff use leucoencephalopathy [Figure 1]. EEG was normal. Serum vasculitis, HIV and VDRL were negative. The cerebrovascular fl uid (CSF) revealed two lymphocytes and raised protein (167 mg/dl; range: 25-45 mg/ dl
Pituitary fossa tomography and air encephalogram: no enlargement of the fossa and no suprasellar ... more Pituitary fossa tomography and air encephalogram: no enlargement of the fossa and no suprasellar extension. * Insulin tolerance test: glucose fell to <25 mg/100 ml. t Measured 20 and 60 minutes after 100 ILg intravenous gonadotrophin releasing hormone (LH/FSH-RH). I Measured by Dr. D. C. Anderson.
Aims To study the association of serum lipids with diabetic retinopathy (DR) in Type 2 diabetic ... more Aims To study the association of serum lipids with diabetic retinopathy (DR) in Type 2 diabetic subjects.Methods Type 2 diabetic subjects (n = 1736) were randomly selected from the Chennai Urban Rural Epidemiology Study (CURES), which was carried out on a representative population of Chennai in South India. DR was diagnosed by retinal colour photography and classified according to the Early Treatment Diabetic Retinopathy Study (ETDRS) grading system. Classification of lipid abnormalities was done according to the National Cholesterol Education Programme–Adult Treatment Panel III (NCEP–ATP III) Guidelines.Results The mean serum cholesterol (P = 0.024), serum triglycerides (P = 0.017) and non‐high‐density lipoprotein (HDL)‐cholesterol (P = 0.025) concentrations were higher in subjects with DR compared with those without DR. Multiple logistic regression analysis revealed that after adjusting for age, gender, duration of diabetes, total cholesterol Standardised regression estimate (S...
The Journal of Clinical Endocrinology & Metabolism, 2000
To compare bilateral inferior petrosal sinus sampling (IPSS) with high dose dexamethasone (HDD) a... more To compare bilateral inferior petrosal sinus sampling (IPSS) with high dose dexamethasone (HDD) and CRH testing (using recently proposed stringent response criteria) in the differential diagnosis of ACTH-dependent Cushing's syndrome, we reviewed 53 consecutive cases. The main analysis was limited to 45 cases with confirmed diagnosis: 44 with pituitary dependency, proven by confirmatory histology and/or significant biochemical improvement after pituitary surgery, and 1 with ectopic ACTH syndrome. After HDD (2 mg every 6 h for 48 h), 21 of the 44 pituitary cases met the stringent more than 90% suppression criterion. Twenty-three of the 44 pituitary cases also underwent CRH testing; 16 of 23 met a stringent response criterion
Indian Journal of Endocrinology and Metabolism, 2019
Introduction: Type 1 diabetes mellitus (T1DM) is associated with various autoimmune disorders lik... more Introduction: Type 1 diabetes mellitus (T1DM) is associated with various autoimmune disorders like celiac disease, thyroid disorder, adrenal failure, etc. However, how common is this association in Indian children is not clearly known. Objective: To assess the prevalence of other coexisting autoimmune disorders in children with T1DM. Materials and Methods: In this cross-sectional study, patients requiring insulin and ketosis-prone diabetic and with history of diabetic ketoacidosis/undetectable fasting C-peptide levels were included. Beside demographic and clinical data, detailed biochemistry evaluations were performed. Celiac disease was diagnosed as per the ESPGHAN diagnostic criteria. ACTH stimulation test was done to confirm the adrenal insufficiency in patients with basal serum cortisol <5 μg/dL. Thyroid function test (TSH) and anti-TPO antibody were assessed in all patients. Screening for other autoimmune disorders was done only when clinically indicated or symptoms or family history was suggestive of presence of such disorder. Results: Among 150 patients enrolled, 64.66% were males and mean age was 13.48 ± 3.29 years (range 3–18 years). Mean age at diagnosis of T1DM was 10.0 ± 3.63 years and duration of diabetes was 3.46 ± 3.18 years. The prevalence of antibodies positive against autoimmune diseases was anti-tTG IgA (20.7%), anti-TPO (33.7%), anti-CCP ab (1.3%), and ANA (0.7%). Significantly higher proportion of females had raised anti-TPO antibodies than males (47.2% vs. 25.8%, P = 0.006). Celiac disease was most common association (24.8%) followed by hypothyroidism (14.1%) and Grave's disease (3.3%). Significantly higher proportion of females had hypothyroidism than males (25.0% vs. 8.2%, respectively, P = 0.005). Prevalence of raised anti-tTG and anti-TPO did not differ significantly by the age (P = 0.841 and P = 0.067) or duration of T1DM (P = 0.493 and P = 0.399). Conclusion: In this part of country, celiac disease, hypothyroidism, and Graves's disease are common associations in children with T1DM.
Congenital hypothyroidism is one of the commonest preventable causes of mental retardation is als... more Congenital hypothyroidism is one of the commonest preventable causes of mental retardation is also the most common congenital endocrine disorder of childhood. The subtlety of clinical features and protective effect of the maternal hormone on fetal brain after crossing the placenta mask the clinical features. The incidence varies from 1 in 4000 to 1 in 1000 in newborn infants in various parts of world and is increasing world wide. Thyroid agenesis remains the most common etiology of CH and other causes are dyshormonogenesis, defects in peripheral thyroid hormone transport, metabolism, or action. CH is usually diagnosed after neonatal screening tests and if treatment started with in few weeks of birth neurodevelopmental outcome is usually normal. Levothyroxine (T4) remains the treatment of choice as most brain T3 is derived from local monodeiodination of T4 and studies have shown normal serum level of T3 in infant treated with T4 alone.
Goiter - Causes and Treatment [Working Title], 2020
Multinodular goiter (MNG) is the most common disorder of the thyroid gland. It is highly endemic ... more Multinodular goiter (MNG) is the most common disorder of the thyroid gland. It is highly endemic in iodine-deficient areas; MNG can be seen in almost all individuals with severe iodine-deficient areas. It starts as a diffuse enlargement of the thyroid gland and ends in a nodular enlarged thyroid. Though MNG can be sporadic, there is a strong correlation between occurrence of MNG and iodine deficiency. The characteristic feature of MNG is its functional and structural heterogeneity. The MNG usually presents as neck swelling; rarely it may produce pressure symptoms, i.e., dyspnea, hoarseness of voice, and dysphagia. It can also present with symptoms of hyperthyroidism particularly in long-standing goiter. Imaging particularly ultrasound is very useful to define characteristic of MNG and surrounding structure. The incidence of malignancy in MNG is 4-14%, and risk factors are family history of thyroid carcinoma, history of neck radiation, prior surgery, and presence of cervical lymphadenopathies. Management of MNG can be done by drugs, surgery, and radioiodine (I-131) depending on results of diagnostic evaluation and associated complications.
Acute suppurative thyroiditis (AST) leading to thyroid abscess is a quite uncommon clinical entit... more Acute suppurative thyroiditis (AST) leading to thyroid abscess is a quite uncommon clinical entity. Both thyroid abscess and AST represent only 0.1%–0.7% of thyroid pathologies which may require surgical management. AST especially affects patients with Hashimoto's thyroiditis or thyroid cancer. In children, AST is associated with the persistence of a canal originating from the 3rd or 4th bronchial pouch that may lead to recurrent thyroid abscess. The left lobe of thyroid gland is more frequently involved. AST can be life threatening if left untreated, resulting in mortality of 12% or higher.
Indian Journal of Endocrinology and Metabolism, 2017
Original Article IntroductIon Type 2 diabetes mellitus (T2DM), a chronic multisystem disorder, is... more Original Article IntroductIon Type 2 diabetes mellitus (T2DM), a chronic multisystem disorder, is known to increase fractures risk. [1] With increasing age, the risk of diabetes and osteoporosis both increases which can lead to greater incidence of fractures. [2,3] Although individuals with T2DM show higher bone mineral density (BMD), a higher fracture risk exists. [1,3,4] Pathophysiologically, impaired bone repair resulting from accumulation of microcracks and increased cortical porosity is reported. [5,6] Osteoporosis is more frequent in postmenopausal women. Postmenopausal women with type 1 diabetes are reported to have 12.25 times increased risk of hip fractures than nondiabetic women. [7] A greater degree of osteopenia and osteoporosis has also been reported in postmenopausal women with T2DM. [8] In these perspectives, we need to look at Indian population in a different way because of cultural diversities. Clinical studies in India have revealed contrasting results to Western studies in that a lower BMD in T2DM was reported by the most, [9-12] whereas only a few found no difference in BMD in T2DM compared to control. [13] To further ascertain the prevalence of osteoporosis in T2DM, we assessed the BMD at spine and hip in otherwise healthy T2DM patients from Jaipur, Rajasthan, India and determined correlation of the osteoporosis with various biochemical parameters. materIalS and methodS This cross-sectional, observational study was conducted at a tertiary care center in a tier I Metro city of Rajasthan, India in T2DM patients. After ethical committee approval, Background and Objective: Type 2 diabetes mellitus (T2DM) may affect bone loss differentially in adult males and postmenopausal females. We evaluated the prevalence of osteoporosis in otherwise healthy adults with T2DM. Materials and Methods: In a cross-sectional study, adults with T2DM, aged 50 years and above, were evaluated for bone mineral density (BMD) using dual-energy X-ray absorptiometry (DXA) scan at spine and hip. T-score of ≤−2.5 was defined as osteoporosis and score −2.49 to −1.0 as osteopenia at either site. Correlation of low BMD with demographic, clinical, and laboratory parameters including serum Vitamin D and serum testosterone (in males) was evaluated. Results: In 200 patients, mean age was 64.5 ± 7.0 years and age differed significantly in males and females (P < 0.0001). Osteoporosis was present in 35.5% adults with T2DM. Significantly greater proportion of females had osteoporosis (49.5% vs. 22.3%, P < 0.0001). Frequency of osteoporosis at spine (33.5%) was higher than the same at hip (13.5%). Compared to males, significantly greater proportion of females had osteoporosis and osteopenia at both spine (P < 0.0001) and hip (P < 0.0001). Among all parameters assessed, a significant positive correlation of T-score at spine and hip was seen with body mass index in both males (r = 0.287, P = 0.003 at spine and r = 0.421, P < 0.0001 at hip) and females (r = 0.291, P = 0.004 at spine and r = 0.280, P = 0.010 at hip). There was no association of Vitamin D deficiency (45.5% patients) with either T-score and presence of osteoporosis either at spine (P = 0.388 and P = 0.177) or hip (P = 0.431 and P = 0.593). Conclusion: Prevalence of osteoporosis in otherwise healthy T2DM was 35.5% with greater prevalence in females than males. Body mass but not Vitamin D or testosterone has an important role in the determination of bone loss in T2DM.
Indian Journal of Endocrinology and Metabolism, 2016
Thyroid dysfunction is a common endocrine disorder. In the US National Health and Nutrition Exami... more Thyroid dysfunction is a common endocrine disorder. In the US National Health and Nutrition Examination Survey, the prevalence of hypothyroidism was 4.6% (0.3 overt and 4.3% subclinical) and the prevalence of hyperthyroidism
Background: Sheehan′s syndrome (SS) or postpartum pituitary necrosis though rare, still remains o... more Background: Sheehan′s syndrome (SS) or postpartum pituitary necrosis though rare, still remains one of the commonest causes of hypopituitarism in the Indian subcontinent. The clinical presentation is often variable with abrupt or insidiously onset pituitary insufficiency after heavy intrapartum or postpartum hemorrhage. Aims: To study the demographic profile, hormonal profile, as well as the quality of life of patients of SS, and the effect of hormonal replacement therapy on outcome. Materials and Methods: All patients previously diagnosed with Sheehan′s syndrome or newly admitted during the study period of 2011-2013 were enrolled in the study after obtaining informed consent. Their clinical, biochemical, hormonal, radiological and bone mineral density (BMD) data were collected. The quality of life (QoL) was assessed using the disease-specific questionnaire both before and after hormone replacement therapy. Result: Twenty three patients were enrolled in the study, the mean age of diagnosis was 43.4 ± 14.2 years; mean diagnostic delay was 13.2 ± 7.4 years. Secondary amenorrhea and lactation failure were the most common clinical presentations. The mean total tetraiodothyronine (T4), peak stimulated cortisol, stimulated growth hormone (GH), and prolactin (PRL) levels were low. The gonadotropins [follicle stimulating hormone (FSH) and luteinizing hormone (LH)] were inappropriately normal in the presence of amenorrhea. Anemia was the most common hematological abnormality seen in 69.5% of patients, while 39.1% of patients had hyponatermia. BMD assessment (n = 23) was suggestive of low bone mass. The QoL improved significantly (P < 0.05) in patients after one year of hormonal replacement therapy. Conclusion: Sheehan′s syndrome resulted in multiple pituitary hormone deficiencies in all patients. Anemia, hyponatremia, and low bone mass were frequently seen in patients with Sheehan′s syndrome. The QoL improved significantly (P < 0.05) after hormonal replacement therapy.
The role of thyroid hormone in pubertal development is complex, with majority of the hypothyroid ... more The role of thyroid hormone in pubertal development is complex, with majority of the hypothyroid patients failing to attain puberty. However, isosexual precocious puberty with delayed bone age has been described in female hypothyroid patients, and is called Van Wyk Grumbach Syndrome (VWGS). The presentation of VWGS in boys is different, with the major presentation being isolated enlargement of the testes, with no signs of virilisation. The cross over action of Thyroid Stimulating Hormone (TSH) on Follicle Stimulating Hormone (FSH) receptor producing Sertoli cell hyperplasia produces testicular enlargement, and lack of action of TSH on Luteinizing hormone (LH) with no Leydig cell hyperplasia explaining lack of virilisation. Delayed bone age and typical features of hypothyroidism help to differentiate this condition from other causes of precocious puberty.
Indian Journal of Endocrinology and Metabolism, 2014
While T1DM has been traditionally seen as a minor concern in the larger picture of pediatric ailm... more While T1DM has been traditionally seen as a minor concern in the larger picture of pediatric ailments, new data reveals that the incidence of T1DM has assumed alarming proportions. It has long been clear that while the disease may be diagnosed at an early age, its impact is not isolated to affl icted children. The direct impact of the disease on the patient is debilitating due to the nature of the disease and lack of proper access to treatment in India. But this impact is further compounded by the utter apathy and often times antipathy, which patients withT1DM have to face. Lack of awareness of the issue in all stakeholders, low access to quality healthcare, patient, physician, and system level barriers to the delivery of optimal diabetes care are some of the factors which hinder successful management of T1DM. The fi rst international consensus meet on diabetes in children was convened with the aim of providing a common platform to all the stakeholders in the management of T1DM, to discuss the academic, administrative and healthcare system related issues. The ultimate aim was to articulate the problems faced by children with diabetes in a way that centralized their position and focused on creating modalities of management sensitive to their needs and aspirations. It was conceptualized to raise a strong voice of advocacy for improving the management of T1DM and ensuring that "No child should die of diabetes". The unique clinical presentations of T1DM coupled with ignorance on the part of the medical community and society in general results in outcomes that are far worse than that seen with T2DM. So there is a need to substantially improve training of HCPs at all levels on this neglected aspect of healthcare.
Pickardt syndrome (Pickardt-Fahlbusch syndrome) is a rare congenital syndrome characterized by te... more Pickardt syndrome (Pickardt-Fahlbusch syndrome) is a rare congenital syndrome characterized by tertiary hypothyroidism caused by the interruption of the portal veins between hypothalamus and adenohypophysis. Typical features of this syndrome are tertiary hypothyroidism with low thyroid stimulating hormone, hyperprolactinemia and other pituitary hormone deficiencies. Pituitary stalk interruption syndrome is characterized by a triad of thin or interrupted pituitary stalk, aplasia or hypoplasia of the anterior pituitary and absent or ectopic posterior pituitary (EPP) seen on magnetic resonance imaging (MRI). It is a congenital anomaly of pituitary whose exact prevalence is unknown. In some cases, it is restricted to EPP or pituitary stalk interruption. We are presenting the case history along with MRI finding of three children&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s who presented with short stature and delayed puberty.
International Journal of Case Reports and Images, 2014
International Journal of Case Reports and Images (IJCRI) is an international, peer reviewed, mont... more International Journal of Case Reports and Images (IJCRI) is an international, peer reviewed, monthly, open access, online journal, publishing high-quality, articles in all areas of basic medical sciences and clinical specialties. Aim of IJCRI is to encourage the publication of new information by providing a platform for reporting of unique, unusual and rare cases which enhance understanding of disease process, its diagnosis, management and clinico-pathologic correlations. IJCRI publishes Review Articles, Case Series, Case Reports, Case in Images, Clinical Images and Letters to Editor.
Hashimoto′s Encephalopathy (HE) can have highly variable neuropsychiatric manifestations, making ... more Hashimoto′s Encephalopathy (HE) can have highly variable neuropsychiatric manifestations, making it difficult to diagnose, and may go unrecognized for a long time. HE is a diagnosis of exclusion and should be kept in mind when evaluating a patient with cognitive dysfunction and high titres of antithyroid antibodies as it responds dramatically to steroids. Steroid responsive myoclonus can be a presentation of HE.
Indian Journal of Endocrinology and Metabolism, 2015
Congenital hypothyroidism (CH) is the one of the most common preventable cause of mental retardat... more Congenital hypothyroidism (CH) is the one of the most common preventable cause of mental retardation. In the majority of patients, CH is caused by an abnormal development of the thyroid gland (thyroid dysgenesis) that is a sporadic disorder and accounts for 85% of cases and the remaining 15% of cases are caused by dyshormonogenesis. The clinical features of congenital hypothyroidism are so subtle that many newborn infants remain undiagnosed at birth and delayed diagnosis leads to the most severe outcome of CH, mental retardation, emphasizing the importance of neonatal screening. Dried capillary blood is used for screening and it is taken from heel prick optimally between 2 and 5 days of age. Blood spot TSH or thyroxine (T4) or both are being used for CH screening in different programs around the world. Neonates with abnormal thyroid screening tests should be recalled immediately for examination and a venipuncture blood sample should be drawn for confirmatory serum testing. Confirmatory serum should be tested for TSH and free T4, or total T4. Serum TSH and T4 undergo dynamic changes in the first weeks of life; it is important to compare serum results with age-normal reference ranges. Treatment should be started promptly and infant should be rendered euthyroid as early as possible, as there is an inverse relationship between intelligence quotient (IQ) and the age at diagnosis. Levothyroxine (l-thyroxine) is the treatment of choice and American academy of pediatrics and European society of pediatric endocrinology recommend 10-15μgm/kg/day as initial dose. The immediate goal of therapy is to normalize T4 within 2 weeks and TSH within one month. The overall goal of treatment is to ensure growth and neurodevelopmental outcomes as close as possible to their genetic potential.
Indian Journal of Endocrinology and Metabolism, 2014
Hashimoto's encephalopathy (HE) is a rare steroid-responsive encephalopathy syndrome, which can h... more Hashimoto's encephalopathy (HE) is a rare steroid-responsive encephalopathy syndrome, which can have highly variable neuropsychiatric manifestations and can go unrecognized for a long time. HE is a diagnosis of exclusion and should be kept in mind when evaluating a patient with a cognitive dysfunction and high titers of anti-thyroid antibodies as it responds dramatically to steroids. Steroid responsive myoclonus can be a presentation of HE.
imaging (MRI) and EEG observations, and response to immunomodulation in three cases of H.E. Case ... more imaging (MRI) and EEG observations, and response to immunomodulation in three cases of H.E. Case Reports Case 1 Mr. R, a 40-year-old-man, presented with progressive myoclonus for 9 months, cognitive decline and visuospatial disorientation for 8 months and dysarthria for 3 months. One month ago, he developed generalized tonic-clonic seizure, irrelevant speech, gait ataxia and startle response. He consumed alcohol occasionally. His general physical examination and vitals were normal. He scored 11/30 on Mini Mental State Examination (MMSE) and a detailed neuropsychological assessment revealed frontal and parietal lobe impairment. He had stimulus-sensitive multifocal myoclonus, limb rigidity and gait ataxia. The routine hemogram and serum biochemistry were normal. Chest X-ray and ultrasonography (USG) (abdomen) were normal. MRI (brain) revealed diff use leucoencephalopathy [Figure 1]. EEG was normal. Serum vasculitis, HIV and VDRL were negative. The cerebrovascular fl uid (CSF) revealed two lymphocytes and raised protein (167 mg/dl; range: 25-45 mg/ dl
Pituitary fossa tomography and air encephalogram: no enlargement of the fossa and no suprasellar ... more Pituitary fossa tomography and air encephalogram: no enlargement of the fossa and no suprasellar extension. * Insulin tolerance test: glucose fell to <25 mg/100 ml. t Measured 20 and 60 minutes after 100 ILg intravenous gonadotrophin releasing hormone (LH/FSH-RH). I Measured by Dr. D. C. Anderson.
Aims To study the association of serum lipids with diabetic retinopathy (DR) in Type 2 diabetic ... more Aims To study the association of serum lipids with diabetic retinopathy (DR) in Type 2 diabetic subjects.Methods Type 2 diabetic subjects (n = 1736) were randomly selected from the Chennai Urban Rural Epidemiology Study (CURES), which was carried out on a representative population of Chennai in South India. DR was diagnosed by retinal colour photography and classified according to the Early Treatment Diabetic Retinopathy Study (ETDRS) grading system. Classification of lipid abnormalities was done according to the National Cholesterol Education Programme–Adult Treatment Panel III (NCEP–ATP III) Guidelines.Results The mean serum cholesterol (P = 0.024), serum triglycerides (P = 0.017) and non‐high‐density lipoprotein (HDL)‐cholesterol (P = 0.025) concentrations were higher in subjects with DR compared with those without DR. Multiple logistic regression analysis revealed that after adjusting for age, gender, duration of diabetes, total cholesterol Standardised regression estimate (S...
The Journal of Clinical Endocrinology & Metabolism, 2000
To compare bilateral inferior petrosal sinus sampling (IPSS) with high dose dexamethasone (HDD) a... more To compare bilateral inferior petrosal sinus sampling (IPSS) with high dose dexamethasone (HDD) and CRH testing (using recently proposed stringent response criteria) in the differential diagnosis of ACTH-dependent Cushing's syndrome, we reviewed 53 consecutive cases. The main analysis was limited to 45 cases with confirmed diagnosis: 44 with pituitary dependency, proven by confirmatory histology and/or significant biochemical improvement after pituitary surgery, and 1 with ectopic ACTH syndrome. After HDD (2 mg every 6 h for 48 h), 21 of the 44 pituitary cases met the stringent more than 90% suppression criterion. Twenty-three of the 44 pituitary cases also underwent CRH testing; 16 of 23 met a stringent response criterion
Indian Journal of Endocrinology and Metabolism, 2019
Introduction: Type 1 diabetes mellitus (T1DM) is associated with various autoimmune disorders lik... more Introduction: Type 1 diabetes mellitus (T1DM) is associated with various autoimmune disorders like celiac disease, thyroid disorder, adrenal failure, etc. However, how common is this association in Indian children is not clearly known. Objective: To assess the prevalence of other coexisting autoimmune disorders in children with T1DM. Materials and Methods: In this cross-sectional study, patients requiring insulin and ketosis-prone diabetic and with history of diabetic ketoacidosis/undetectable fasting C-peptide levels were included. Beside demographic and clinical data, detailed biochemistry evaluations were performed. Celiac disease was diagnosed as per the ESPGHAN diagnostic criteria. ACTH stimulation test was done to confirm the adrenal insufficiency in patients with basal serum cortisol <5 μg/dL. Thyroid function test (TSH) and anti-TPO antibody were assessed in all patients. Screening for other autoimmune disorders was done only when clinically indicated or symptoms or family history was suggestive of presence of such disorder. Results: Among 150 patients enrolled, 64.66% were males and mean age was 13.48 ± 3.29 years (range 3–18 years). Mean age at diagnosis of T1DM was 10.0 ± 3.63 years and duration of diabetes was 3.46 ± 3.18 years. The prevalence of antibodies positive against autoimmune diseases was anti-tTG IgA (20.7%), anti-TPO (33.7%), anti-CCP ab (1.3%), and ANA (0.7%). Significantly higher proportion of females had raised anti-TPO antibodies than males (47.2% vs. 25.8%, P = 0.006). Celiac disease was most common association (24.8%) followed by hypothyroidism (14.1%) and Grave's disease (3.3%). Significantly higher proportion of females had hypothyroidism than males (25.0% vs. 8.2%, respectively, P = 0.005). Prevalence of raised anti-tTG and anti-TPO did not differ significantly by the age (P = 0.841 and P = 0.067) or duration of T1DM (P = 0.493 and P = 0.399). Conclusion: In this part of country, celiac disease, hypothyroidism, and Graves's disease are common associations in children with T1DM.
Congenital hypothyroidism is one of the commonest preventable causes of mental retardation is als... more Congenital hypothyroidism is one of the commonest preventable causes of mental retardation is also the most common congenital endocrine disorder of childhood. The subtlety of clinical features and protective effect of the maternal hormone on fetal brain after crossing the placenta mask the clinical features. The incidence varies from 1 in 4000 to 1 in 1000 in newborn infants in various parts of world and is increasing world wide. Thyroid agenesis remains the most common etiology of CH and other causes are dyshormonogenesis, defects in peripheral thyroid hormone transport, metabolism, or action. CH is usually diagnosed after neonatal screening tests and if treatment started with in few weeks of birth neurodevelopmental outcome is usually normal. Levothyroxine (T4) remains the treatment of choice as most brain T3 is derived from local monodeiodination of T4 and studies have shown normal serum level of T3 in infant treated with T4 alone.
Goiter - Causes and Treatment [Working Title], 2020
Multinodular goiter (MNG) is the most common disorder of the thyroid gland. It is highly endemic ... more Multinodular goiter (MNG) is the most common disorder of the thyroid gland. It is highly endemic in iodine-deficient areas; MNG can be seen in almost all individuals with severe iodine-deficient areas. It starts as a diffuse enlargement of the thyroid gland and ends in a nodular enlarged thyroid. Though MNG can be sporadic, there is a strong correlation between occurrence of MNG and iodine deficiency. The characteristic feature of MNG is its functional and structural heterogeneity. The MNG usually presents as neck swelling; rarely it may produce pressure symptoms, i.e., dyspnea, hoarseness of voice, and dysphagia. It can also present with symptoms of hyperthyroidism particularly in long-standing goiter. Imaging particularly ultrasound is very useful to define characteristic of MNG and surrounding structure. The incidence of malignancy in MNG is 4-14%, and risk factors are family history of thyroid carcinoma, history of neck radiation, prior surgery, and presence of cervical lymphadenopathies. Management of MNG can be done by drugs, surgery, and radioiodine (I-131) depending on results of diagnostic evaluation and associated complications.
Acute suppurative thyroiditis (AST) leading to thyroid abscess is a quite uncommon clinical entit... more Acute suppurative thyroiditis (AST) leading to thyroid abscess is a quite uncommon clinical entity. Both thyroid abscess and AST represent only 0.1%–0.7% of thyroid pathologies which may require surgical management. AST especially affects patients with Hashimoto's thyroiditis or thyroid cancer. In children, AST is associated with the persistence of a canal originating from the 3rd or 4th bronchial pouch that may lead to recurrent thyroid abscess. The left lobe of thyroid gland is more frequently involved. AST can be life threatening if left untreated, resulting in mortality of 12% or higher.
Indian Journal of Endocrinology and Metabolism, 2017
Original Article IntroductIon Type 2 diabetes mellitus (T2DM), a chronic multisystem disorder, is... more Original Article IntroductIon Type 2 diabetes mellitus (T2DM), a chronic multisystem disorder, is known to increase fractures risk. [1] With increasing age, the risk of diabetes and osteoporosis both increases which can lead to greater incidence of fractures. [2,3] Although individuals with T2DM show higher bone mineral density (BMD), a higher fracture risk exists. [1,3,4] Pathophysiologically, impaired bone repair resulting from accumulation of microcracks and increased cortical porosity is reported. [5,6] Osteoporosis is more frequent in postmenopausal women. Postmenopausal women with type 1 diabetes are reported to have 12.25 times increased risk of hip fractures than nondiabetic women. [7] A greater degree of osteopenia and osteoporosis has also been reported in postmenopausal women with T2DM. [8] In these perspectives, we need to look at Indian population in a different way because of cultural diversities. Clinical studies in India have revealed contrasting results to Western studies in that a lower BMD in T2DM was reported by the most, [9-12] whereas only a few found no difference in BMD in T2DM compared to control. [13] To further ascertain the prevalence of osteoporosis in T2DM, we assessed the BMD at spine and hip in otherwise healthy T2DM patients from Jaipur, Rajasthan, India and determined correlation of the osteoporosis with various biochemical parameters. materIalS and methodS This cross-sectional, observational study was conducted at a tertiary care center in a tier I Metro city of Rajasthan, India in T2DM patients. After ethical committee approval, Background and Objective: Type 2 diabetes mellitus (T2DM) may affect bone loss differentially in adult males and postmenopausal females. We evaluated the prevalence of osteoporosis in otherwise healthy adults with T2DM. Materials and Methods: In a cross-sectional study, adults with T2DM, aged 50 years and above, were evaluated for bone mineral density (BMD) using dual-energy X-ray absorptiometry (DXA) scan at spine and hip. T-score of ≤−2.5 was defined as osteoporosis and score −2.49 to −1.0 as osteopenia at either site. Correlation of low BMD with demographic, clinical, and laboratory parameters including serum Vitamin D and serum testosterone (in males) was evaluated. Results: In 200 patients, mean age was 64.5 ± 7.0 years and age differed significantly in males and females (P < 0.0001). Osteoporosis was present in 35.5% adults with T2DM. Significantly greater proportion of females had osteoporosis (49.5% vs. 22.3%, P < 0.0001). Frequency of osteoporosis at spine (33.5%) was higher than the same at hip (13.5%). Compared to males, significantly greater proportion of females had osteoporosis and osteopenia at both spine (P < 0.0001) and hip (P < 0.0001). Among all parameters assessed, a significant positive correlation of T-score at spine and hip was seen with body mass index in both males (r = 0.287, P = 0.003 at spine and r = 0.421, P < 0.0001 at hip) and females (r = 0.291, P = 0.004 at spine and r = 0.280, P = 0.010 at hip). There was no association of Vitamin D deficiency (45.5% patients) with either T-score and presence of osteoporosis either at spine (P = 0.388 and P = 0.177) or hip (P = 0.431 and P = 0.593). Conclusion: Prevalence of osteoporosis in otherwise healthy T2DM was 35.5% with greater prevalence in females than males. Body mass but not Vitamin D or testosterone has an important role in the determination of bone loss in T2DM.
Indian Journal of Endocrinology and Metabolism, 2016
Thyroid dysfunction is a common endocrine disorder. In the US National Health and Nutrition Exami... more Thyroid dysfunction is a common endocrine disorder. In the US National Health and Nutrition Examination Survey, the prevalence of hypothyroidism was 4.6% (0.3 overt and 4.3% subclinical) and the prevalence of hyperthyroidism
Background: Sheehan′s syndrome (SS) or postpartum pituitary necrosis though rare, still remains o... more Background: Sheehan′s syndrome (SS) or postpartum pituitary necrosis though rare, still remains one of the commonest causes of hypopituitarism in the Indian subcontinent. The clinical presentation is often variable with abrupt or insidiously onset pituitary insufficiency after heavy intrapartum or postpartum hemorrhage. Aims: To study the demographic profile, hormonal profile, as well as the quality of life of patients of SS, and the effect of hormonal replacement therapy on outcome. Materials and Methods: All patients previously diagnosed with Sheehan′s syndrome or newly admitted during the study period of 2011-2013 were enrolled in the study after obtaining informed consent. Their clinical, biochemical, hormonal, radiological and bone mineral density (BMD) data were collected. The quality of life (QoL) was assessed using the disease-specific questionnaire both before and after hormone replacement therapy. Result: Twenty three patients were enrolled in the study, the mean age of diagnosis was 43.4 ± 14.2 years; mean diagnostic delay was 13.2 ± 7.4 years. Secondary amenorrhea and lactation failure were the most common clinical presentations. The mean total tetraiodothyronine (T4), peak stimulated cortisol, stimulated growth hormone (GH), and prolactin (PRL) levels were low. The gonadotropins [follicle stimulating hormone (FSH) and luteinizing hormone (LH)] were inappropriately normal in the presence of amenorrhea. Anemia was the most common hematological abnormality seen in 69.5% of patients, while 39.1% of patients had hyponatermia. BMD assessment (n = 23) was suggestive of low bone mass. The QoL improved significantly (P < 0.05) in patients after one year of hormonal replacement therapy. Conclusion: Sheehan′s syndrome resulted in multiple pituitary hormone deficiencies in all patients. Anemia, hyponatremia, and low bone mass were frequently seen in patients with Sheehan′s syndrome. The QoL improved significantly (P < 0.05) after hormonal replacement therapy.
The role of thyroid hormone in pubertal development is complex, with majority of the hypothyroid ... more The role of thyroid hormone in pubertal development is complex, with majority of the hypothyroid patients failing to attain puberty. However, isosexual precocious puberty with delayed bone age has been described in female hypothyroid patients, and is called Van Wyk Grumbach Syndrome (VWGS). The presentation of VWGS in boys is different, with the major presentation being isolated enlargement of the testes, with no signs of virilisation. The cross over action of Thyroid Stimulating Hormone (TSH) on Follicle Stimulating Hormone (FSH) receptor producing Sertoli cell hyperplasia produces testicular enlargement, and lack of action of TSH on Luteinizing hormone (LH) with no Leydig cell hyperplasia explaining lack of virilisation. Delayed bone age and typical features of hypothyroidism help to differentiate this condition from other causes of precocious puberty.
Indian Journal of Endocrinology and Metabolism, 2014
While T1DM has been traditionally seen as a minor concern in the larger picture of pediatric ailm... more While T1DM has been traditionally seen as a minor concern in the larger picture of pediatric ailments, new data reveals that the incidence of T1DM has assumed alarming proportions. It has long been clear that while the disease may be diagnosed at an early age, its impact is not isolated to affl icted children. The direct impact of the disease on the patient is debilitating due to the nature of the disease and lack of proper access to treatment in India. But this impact is further compounded by the utter apathy and often times antipathy, which patients withT1DM have to face. Lack of awareness of the issue in all stakeholders, low access to quality healthcare, patient, physician, and system level barriers to the delivery of optimal diabetes care are some of the factors which hinder successful management of T1DM. The fi rst international consensus meet on diabetes in children was convened with the aim of providing a common platform to all the stakeholders in the management of T1DM, to discuss the academic, administrative and healthcare system related issues. The ultimate aim was to articulate the problems faced by children with diabetes in a way that centralized their position and focused on creating modalities of management sensitive to their needs and aspirations. It was conceptualized to raise a strong voice of advocacy for improving the management of T1DM and ensuring that "No child should die of diabetes". The unique clinical presentations of T1DM coupled with ignorance on the part of the medical community and society in general results in outcomes that are far worse than that seen with T2DM. So there is a need to substantially improve training of HCPs at all levels on this neglected aspect of healthcare.
Pickardt syndrome (Pickardt-Fahlbusch syndrome) is a rare congenital syndrome characterized by te... more Pickardt syndrome (Pickardt-Fahlbusch syndrome) is a rare congenital syndrome characterized by tertiary hypothyroidism caused by the interruption of the portal veins between hypothalamus and adenohypophysis. Typical features of this syndrome are tertiary hypothyroidism with low thyroid stimulating hormone, hyperprolactinemia and other pituitary hormone deficiencies. Pituitary stalk interruption syndrome is characterized by a triad of thin or interrupted pituitary stalk, aplasia or hypoplasia of the anterior pituitary and absent or ectopic posterior pituitary (EPP) seen on magnetic resonance imaging (MRI). It is a congenital anomaly of pituitary whose exact prevalence is unknown. In some cases, it is restricted to EPP or pituitary stalk interruption. We are presenting the case history along with MRI finding of three children&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s who presented with short stature and delayed puberty.
International Journal of Case Reports and Images, 2014
International Journal of Case Reports and Images (IJCRI) is an international, peer reviewed, mont... more International Journal of Case Reports and Images (IJCRI) is an international, peer reviewed, monthly, open access, online journal, publishing high-quality, articles in all areas of basic medical sciences and clinical specialties. Aim of IJCRI is to encourage the publication of new information by providing a platform for reporting of unique, unusual and rare cases which enhance understanding of disease process, its diagnosis, management and clinico-pathologic correlations. IJCRI publishes Review Articles, Case Series, Case Reports, Case in Images, Clinical Images and Letters to Editor.
Hashimoto′s Encephalopathy (HE) can have highly variable neuropsychiatric manifestations, making ... more Hashimoto′s Encephalopathy (HE) can have highly variable neuropsychiatric manifestations, making it difficult to diagnose, and may go unrecognized for a long time. HE is a diagnosis of exclusion and should be kept in mind when evaluating a patient with cognitive dysfunction and high titres of antithyroid antibodies as it responds dramatically to steroids. Steroid responsive myoclonus can be a presentation of HE.
Indian Journal of Endocrinology and Metabolism, 2015
Congenital hypothyroidism (CH) is the one of the most common preventable cause of mental retardat... more Congenital hypothyroidism (CH) is the one of the most common preventable cause of mental retardation. In the majority of patients, CH is caused by an abnormal development of the thyroid gland (thyroid dysgenesis) that is a sporadic disorder and accounts for 85% of cases and the remaining 15% of cases are caused by dyshormonogenesis. The clinical features of congenital hypothyroidism are so subtle that many newborn infants remain undiagnosed at birth and delayed diagnosis leads to the most severe outcome of CH, mental retardation, emphasizing the importance of neonatal screening. Dried capillary blood is used for screening and it is taken from heel prick optimally between 2 and 5 days of age. Blood spot TSH or thyroxine (T4) or both are being used for CH screening in different programs around the world. Neonates with abnormal thyroid screening tests should be recalled immediately for examination and a venipuncture blood sample should be drawn for confirmatory serum testing. Confirmatory serum should be tested for TSH and free T4, or total T4. Serum TSH and T4 undergo dynamic changes in the first weeks of life; it is important to compare serum results with age-normal reference ranges. Treatment should be started promptly and infant should be rendered euthyroid as early as possible, as there is an inverse relationship between intelligence quotient (IQ) and the age at diagnosis. Levothyroxine (l-thyroxine) is the treatment of choice and American academy of pediatrics and European society of pediatric endocrinology recommend 10-15μgm/kg/day as initial dose. The immediate goal of therapy is to normalize T4 within 2 weeks and TSH within one month. The overall goal of treatment is to ensure growth and neurodevelopmental outcomes as close as possible to their genetic potential.
Indian Journal of Endocrinology and Metabolism, 2014
Hashimoto's encephalopathy (HE) is a rare steroid-responsive encephalopathy syndrome, which can h... more Hashimoto's encephalopathy (HE) is a rare steroid-responsive encephalopathy syndrome, which can have highly variable neuropsychiatric manifestations and can go unrecognized for a long time. HE is a diagnosis of exclusion and should be kept in mind when evaluating a patient with a cognitive dysfunction and high titers of anti-thyroid antibodies as it responds dramatically to steroids. Steroid responsive myoclonus can be a presentation of HE.
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