Introduction: Children with congenital adrenal hyperplasia (CAH) provide us an opportunity to study the clinical effects of androgen excess in humans. We studied the sequence of pubertal development in girls with congenital adrenal hyperplasia initiated on treatment at different ages, to assess the effects of androgen exposure on the Hypothalamic-Pituitary-Ovarian (HPO) axis. Materials and Methods: Girls more than 18 years of age, with CAH, on follow-up at this hospital were the subjects for this study. Details of history, physical findings, laboratory evaluation, and medication were noted from their case records and verified from the patients and their / parents, in addition to assessment of their present health status. Result: We studied 24 patients of classical CAH (SW-2, SV-22, average age - 24.5 ± 6.6 years). All had varying degrees of genital ambiguity (Prader stage 3 (n = 13), Prader stage 2 (n = 10), Prader stage 1 (n = 1). Among them were13 girls, who were started on steroids after eight years of age. Girls who received treatment from infancy and early childhood had normal pubertal development (mean age at menarche 11.4 ± 1.7 years). Hirsutism was not a problem among them. Untreated children had progressive clitoral enlargement throughout childhood, developed pubic hair at around three to six years of age, and facial hair between nine and eleven years. Plasma testosterone ranged from 3 to 6 ng / ml prior to treatment. Six of the 13 untreated CAH girls had subtle breast development starting at ages 11 - 16 years and three had spontaneous infrequent vaginal bleeding starting at ages 11 - 17. Steroid supplementation initiated pubertal changes in older girls in two-to-six months' time. Conclusion: There was a delay in HPO axis maturation (as evidenced by delayed pubertal development) in the absence of treatment in girls with CAH. This could be corrected with steroid supplementation.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Precocious puberty defined as an onset of puberty below eight years in girls and nine years in boys, has an incidence of approximately 1 / 5,000 - 1 / 10,000 subjects with a female / male ratio of 20: 1. It is etiologically classified broadly as central and peripheral. We present to you a case of isosexual (central), precocious puberty in a 16-month-old girl, who was symptomatic since the age of six months, and was later, diagnosed to have hypothalamic hamartoma. It is one of the earliest case records ever in the medical literature of menarche, at an extremely early age (six-month-old child) secondary to a central cause.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Background: Diabetes Mellitus is the commonest endocrine-metabolic disorder in Nigeria similar to the experience in other parts of the world. The aim was to assess the clinical and laboratory profile, and evaluate the quality of care of Nigerian diabetics with a view to planning improved diabetes care. Materials and Methods: In a multicenter study across seven tertiary health centers in Nigeria, the clinical and laboratory parameters of diabetic out-patients were evaluated. Clinical parameters studied include type of diabetes, anthropometry, and blood pressure (BP) status, chronic complications of diabetes, and treatment types. Laboratory data assessed included fasting plasma glucose (FPG), 2-h post-prandial (2-HrPP) glucose, glycated hemoglobin (HbA1c), urinalysis, serum lipids, electrolytes, urea, and creatinine. Results: A total of 531 patients, 209 (39.4%) males and 322 (60.6%) females enrolled. The mean age of the patients was 57.1 ± 12.3 years with the mean duration of diabetes of 8.8 ± 6.6 years. Majority (95.4%) had type 2 diabetes mellitus (DM) compared to type 1 DM (4.6%), with P < 0.001. The mean FPG, 2-HrPP glucose, and HbA1c were 8.1 ± 3.9 mmol/L, 10.6 ± 4.6 mmol/L, and 8.3 ± 2.2%, respectively. Only 170 (32.4%) and 100 (20.4%) patients achieved the ADA and IDF glycemic targets, respectively. Most patients (72.8%) did not practice self-monitoring of blood glucose. Hypertension was found in 322 (60.9%), with mean systolic BP 142.0 ± 23.7 mmHg, and mean diastolic BP 80.7 ± 12.7 mmHg. Diabetic complications found were peripheral neuropathy (59.2%), retinopathy (35.5%), cataracts (25.2%), cerebrovascular disease (4.7%), diabetic foot ulcers (16.0%), and nephropathy (3.2%). Conclusion: Most Nigerian diabetics have suboptimal glycemic control, are hypertensives, and have chronic complications of DM. Improved quality of care and treatment to target is recommended to reduce diabetes-related morbidity and mortality.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Fasting during Ramadan, the holy month of Islam, is mandatory for all healthy adult Muslims. It is estimated that there are 1.1-1.5 billion Muslims worldwide, comprising 18-25% of the world population. About 62% of the world's Muslim population resides in Asia. Women comprise approximately 50% of this population. There is great religious fervor and enthusiasm in the majority of Muslims the world over for observing the religious fasting. Many of the Muslim women perhaps due to the family and societal pressures or lack of proper information hesitate and fail to avail themselves of the generous provisions of temporary or permanent exemptions from fasting available in Islam. It is therefore important that medical professionals as well as the general population be aware of potential risks that may be associated with fasting during Ramadan. This familiarity and knowledge is as important in South Asia and the Middle East as it is in Europe, North America, New Zealand, and Australia. There has not yet been any statement of consensus regarding women's health issues during Ramadan, namely menstruation, sexual obligations of married life, pregnancy, and lactation. This document aims to put forward some of the general guidelines for these issues especially for the South Asian Muslim women.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Adrenocortical carcinomas are rare tumors with an incidence of one to two cases per million population and are still more rarer in the pediatric age group. Adrenocortical carcinomas can be functional or may be unassociated with syndromes of hormone overproduction. It is very important to differentiate an adrenocortical adenoma from a carcinoma, as both share a large number of phenotypic features, and assess their prognosis, as adrenocortical carcinoma may need an adjuvant therapy. In this communication, we describe the case of a two-year-old boy, who presented with iso-sexual precocious puberty, having features of virilization, which included growth of facial and pubic hair, deepening of voice, and penile growth.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Glycated hemoglobin (HbA1c) is an important indicator of glycemic control in diabetes mellitus, based on which important diagnostic and therapeutic decisions are routinely made. However, there are several situations in which the level of HbA1c may not faithfully reflect the glycemic control in a given patient. Important among these is the use of certain non-diabetic medications, which can affect the HbA1c levels in different ways. This review focuses on the non-diabetic medications which can inappropriately raise or lower the HbA1c levels, and the postulated mechanisms for the same.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]

Necrobiosis lipoidica diabeticorum (NLD) is a rare chronic and granulomatous skin disorder that affects 0.3% of diabetic patients. Although the etiology and pathogenesis of NLD is still controversial, it is thought that microangiopathy has an important role. The legs are the most common site for NLD, but involvement of other areas such as the abdomen, upper extremities and scalp has been reported. There is no rational therapy. However, benefit has been reported from different treatment regimens such as drugs acting on the hemostatic mechanisms, corticosteroid therapy (topical, intralesional and systemic), enhancers of wound healing, surgery and immunomodulating therapies (including photochemotherapy). We report a 59-year-old female, who was a diabetic patient with multiple, disseminated lesions on the legs, which tended to disappear as the glycemic control was achieved. Hereby, we also review the existing literature for the evolving aspects of etiopathogenesis and treatment.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Context: Studies on the human interleukin 1 receptor antagonist (IL-1RA) gene polymorphism have provided conflicting data regarding the bone mass and quality. Aim and Design: The objective of this case-control study was to investigate the association between the forearm bone mineral density (BMD) and the IL1RA gene polymorphisms. Materials and Methods: A total of 400 postmenopausal Bulgarian women participated in this study. BMD was measured at the forearm by X-ray absorptiometry on a DTX-100 device (Osteometer Meditech, USA). A PCR product was isolated. The alleles were scored according to their length: A1 - 410 bp - 4 repeats; A2 - 240 bp - 2 repeats; A3 - 500 bp - 5 repeats; A4 - 325 bp - 3 repeats; A5 - 595 bp - 6 repeats. All analyses were evaluated for statistical significance (χ² -test and T-test). Results: Four alleles were observed - A1, A2, A3, and A4. The A1A1 genotype was more common in cases with low BMD than in controls with normal BMD (95% vs. 90%, χ² P < 0.01). The A2A2 genotype was equally distributed among cases and controls (both 5%). The other two genotypes (A3A3 and A4A4) as well as A1A3 were present only in controls with normal BMD. The A2A2 genotype was associated with higher BMD and the A1A1 - with lower BMD at both forearm sites. The odds ratio for low BMD in the presence of the A1A1 genotype was 2.11. The etiological factor reflecting the association between the polymorphism and the disease was 0.50. In our study sample the IL1RA genetic polymorphisms were associated with the forearm BMD. Conclusion: This genetic polymorphism may become a useful genetic marker for the study of osteoporosis.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]

It is obligatory for all adult Muslims to observe fast during the holy month of Ramadan, but sick individuals including those with diabetes mellitus are exempted from the duty of fasting. Specific medical advice must be provided to individual patients concerning the potential risks they must accept if they decide to fast. Any alteration in medications deemed necessary to provide an effective and safe antidiabetic regimen should be instituted well before the start of Ramadan. Diet-controlled patients and those well controlled on insulin sensitizers have low risk of hypoglycemia and may safely fast with some modification in the timing of the doses. Newer generation sulfonylureas (gliclazide MR and glimepiride) have reasonable safety profile during Ramadan fasting and are economical options for a large number of diabetics worldwide, especially in the developing countries; older, long acting sulfonylureas like glibenclamide and chlorpropamide should be avoided during fasting. Oral DPP-IV inhibitors are important substitutes to sulfonylureas for patients with diabetes mellitus during fasting owing to their glucose-dependent mechanism of action, efficacy, and tolerability. This group of drugs causes a moderate A1c reduction, are weight neutral, and have a very low risk of hypoglycemia. Short-acting insulin secretagogues are an option in the subset of fasting diabetic patients who have predominantly post-prandial hyperglycemia.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

A person with diabetes mellitus (DM) has every right to perform the ritual of fasting during Ramadan. In daily practice we come across both type 1 and type 2 DM who wish to fast. This Consensus Statement describes the pre-Ramadan assessment, planning, prescription, management, and monitoring of patients on insulin, who wish to fast.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

In the month of Ramadan, patients with thyroid diseases, most of the time, do not need treatment adjustments and can fast safely without any health hazards. Patients with hypothyroidism taking thyroxine can take their tablets on an empty stomach at bedtime instead of half an hour before Sehr. Patients with hyperthyroidism, on methimazole/carbimazole can continue their dose in once or twice daily regimes, while those on propylthiouracil need to be switched. Hyperthyroid patients with severe symptoms should start treatment immediately and can avoid fast for few days after a consultation with their religious scholar.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Aim: Combination of laparoscopic ileal interposition (II) with sleeve gastrectomy (SG) is an upcoming procedure, which offers good metabolic improvement and weight reduction without causing significant malabsorption. The objective of this study was to evaluate the results of this novel procedure for control of type 2 diabetes, obesity, hypertension, and related metabolic abnormalities. Materials and Methods: The II and SG was performed in 43 patients (M:F = 25:18) from February 2008. Participants had a mean age of 47.2 ± 8.2 years (range 29-66 years), mean duration of diabetes of 10.1 ± 9.2 years (range 1-32 years), and mean preoperative body mass index (BMI) of 33.2 ± 7.8 kg/m2. All patients had poorly controlled type 2 diabetes mellitus (DM) [mean glycated hemoglobin (HbA1C) 9.6 ± 2.1%] despite use of oral hypoglycemic agents (OHAs) and/or insulin. Thirty (70%) patients had hypertension, 20 (46%) had dyslipidemia, and 18 (42%) had significant microalbuminuria. The primary outcome was remission of diabetes (HbA1C < 6.5% without OHAs/insulin) and the secondary outcomes were reduction in antidiabetic agent requirement and components of metabolic syndrome. Results: Mean follow-up was for 20.2 ± 8.6 months (range 4-40 months). Postoperatively, glycemic parameters (fasting and post-lunch blood sugar, HbA1C improved in all patients (P < 0.05) at all intervals. Twenty (47%) patients had remission in diabetes and the remaining patients showed significantly decreased OHA requirement. All patients had weight loss between 15 and 30% (P < 0.05). Twenty-seven (90%) patients had remission in hypertension. At 3 years, the mean fall in HbA1C (34%) was more than reduction in BMI (25%). There was a declining trend in lipids and microalbuminuria postoperatively, though it was significant for microalbuminuria only. Conclusions: The laparoscopic II with SG seems to be a promising procedure for control of type 2 DM, hypertension, weight reduction, and associated metabolic abnormalities. A multicenter study with larger number of patients and a longer follow-up period is needed to substantiate our preliminary findings.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Aims: The aim was to assess the dietary pattern during Ramadan season among type 2 diabetic Muslim subjects who underwent fasting and intensive dietary counseling. Materials and Methods: The study was conducted among 70 Muslim subjects with type 2 diabetes mellitus who undertook fasting during Ramadan and was part of a randomized control trial using pioglitazone published previously. All subjects were subjected to a dietary assessment and counseling at three stages, i.e., initiation of the study, mid-Ramadan and post-Ramadan, by a trained dietician. Dietary assessment was done by the 24-hour dietary recall method and the food frequency questionnaire. Diabetic diet sheets were dispensed to subjects based on their body mass index (BMI), daily activity, and needs. Results: The mean caloric intake between pre-Ramadan (before fasting) and mid-Ramadan (15 days after fasting) were 1506.80 kcal and 1614.29 (P = 0.001) respectively. The distribution of active components pre and during Ramadan were: carbohydrates (g) 260.76 and 265.35 g (P = 0.001), proteins (g) 43.64 and 46.19 (P = 0.001) and fat (g) was 32.88 and 44.16 (P = 0.0001) respectively. The percentage of energy from dietary carbohydrate prior to fasting (64.11 ± 6.73) and during fasting (68.41 ± 4.41) remained almost unchanged but statistically significant when compared at different intervals before and during fasting. Fat intake increased significantly during fasting (P = < 0.001). Conclusions: The dietary composition in a type 2 diabetic Muslim population who undertook fasting during Ramadan showed a mean increase in consumption of all components of diet during the period of fasting. Nutritional compliance during such a time seems to be difficult and warrants repeated counseling and regular follow-up to achieve targets.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Context: Hypothyroidism is a common public health problem in India. With iodine sufficiency, autoimmune thyroiditis is becoming the most important etiology of hypothyroidism. Often, thyroiditis is associated with other systemic autoimmune diseases. Aims: We undertook thisobservational study to find the prevalence of systemic lupus erythematosus (SLE) amongst the hypothyroid patients at our Institution. Settings and Design: This is probably the first study of its kind from India. Materials and Methods: 185 patients with diagnosed hypothyroidism were included and screening for SLE was done by standard epidemiological criteria. Majority of the patients (63.8%) were young adults (20-40 years). Statistical Analysis Used: Two by two contingency tables were analyzed by Chi-square test or Fisher's exact test as needed. Logistic regression model was used considering the presence of SLE as a dependent variable. Results: Eleven (5.94%) patients were found to have SLE. However, anti nuclear factor was positive in 145 cases (78.4%). Of the patients with SLE, 8 (72.7%) were found to be anti TPO positive, but the titers of ANF and anti TPO did not correlate. Presence of discoid rash, haematological criteria and presence of antibodies like anti-dsDNA were significantly correlated with the presence of SLE in hypothyroid patients. Presence of ANF was also correlated with the grade of goiter (r=0.62; P<0.05). Also four patients with SLE had a positive family history (OR=9.37). Logistic regression model showed anti-TPO has OR=1.54 (P=0.02) for the development of SLE. Conclusions: Prevalence of SLE in hypothyroid patients is high compared to the general population, especially, as thyroiditis is very common in SLE.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Background : Thyroid hormones affect bone remodeling in patients with thyroid disease by acting directly or indirectly on bone cells. In view of limited information on correlation of thyroid function with bone mineral density (BMD) in euthyroid subjects, we undertook this study to evaluate the correlation between thyroid function with BMD in subjects with normal thyroid function and subclinical hypothyroidism. Material and Methods : A total of 1290 subjects included in this cross sectional study, were divided in Group-1 with normal thyroid function and Group-2 with subclinical hypothyroidism. Fasting blood samples were drawn for the estimation of serum 25(OH)D, intact parathyroid hormone, total and ionized calcium, inorganic phosphorus, and alkaline phosphatase. BMD at lumbar spine, femur, and forearm was measured. Results : BMD at all sites (radius, femur, and spine) were comparable in both groups. There was no difference in BMD when subjects were divided in tertiles of TSH in either group. In group-1, FT4 and TSH were positively associated with BMD at 33% radius whereas FT3 was negatively associated with BMD at femoral neck in multiple regression analysis after adjustment for age, sex, BMI, 25(OH)D and PTH levels. In group-2, there was no association observed between TSH and BMD at any site. Amongst all study subjects FT4 and FT3 were positively correlated with BMD at lumbar spine and radius respectively among all subjects. Conclusion: TSH does not affect BMD in euthyroid subjects and subjects with subclinical hypothyroidism. Thyroid hormones appear to have more pronounced positive effect on cortical than trabecular bone in euthyroid subjects.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Thyrotoxicosis due to functioning metastases from thyroid cancer is rare. It also presents a therapeutic challenge, as both the metastatic cancer and thyrotoxicosis need to be treated. We present here two cases of thyrotoxicosis which on a routine ^99m Tc-pertechnetate thyroid scan showed extrathyroidal foci of uptake. Two patients who initially presented with thyrotoxicosis underwent a routine thyroid scan. Abnormal uptake in the shoulder was incidentally noted, which prompted us to do a whole body pertechnetate scan in the same sitting, which revealed extensive hyperfunctioning metastases in the lungs and bones. We also discuss the 'Flip Flop' phenomenon in thyroid cancer, which was seen in our case. This report emphasizes the importance of evaluating the abnormal foci of uptake seen on a routine thyroid scan.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Majority of physicians are of the opinion that Ramadan fasting is acceptable for well-balanced type 2 patients conscious of their disease and compliant with their diet and drug intake. Fasting during Ramadan for patients with diabetes carries a risk of an assortment of complications. Islamic rules allow patients not to fast. However, if patient with diabetes wish to fast, it is necessary to advice them to undertake regular monitoring of blood glucose levels several times a day, to reduce the risk of hypoglycemia during day time fasting or hyperglycemia during the night. Patient with type 1 diabetes who fast during Ramadan may be better managed with fast-acting insulin. They should have basic knowledge of carbohydrate metabolism, the standard principles of diabetes care, and pharmacology of various antidiabetic drugs. This Consensus Statement describes the management of the various diabetic emergencies that may occur during Ramadan.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Adrenal insufficiency is a life-threatening event, so it is recommended for patients with known adrenal insufficiency to be properly educated regarding sick-day management. In the month of Ramadan, people refrain from eating and drinking during daylight hours. It is very important for patients with adrenal insufficiency, who wish to keep a fast, to be well aware of the disease, the suitable drug to be used for that particular period, warning signs, sick-day management, physical activity, and dietary limits. This article describes guidelines for the sick-day management of patients with adrenal insufficiency, in the month of Ramadan.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Thyroid anatomy and physiology change in the elderly with age-related fibrosis and atrophy in the thyroid gland and changes in thyroid hormones. The incidence of thyroid nodules increases with age, making the thyroid more nodular. Hypothyroidism is common in the elderly and, if untreated, is associated with significant morbidity. Elderly patients are sensitive to iatrogenic hyperthyroidism, especially with preexisting cardiac disease. Hence, treatment of hypothyroidism should be individualized and should be started with low doses and titrated according to response. Hyperthyroidism, although less common in the elderly if present, is associated with significant cardiac morbidity and mortality. Radioiodine therapy is considered a safe primary treatment in the elderly with hyperthyroidism. Management of subclinical hypo- and hyperthyroidism is still controversial. The incidence of thyroid tumors increase with age. Thyroid malignancy in the elderly is considered as a more advanced disease compared with the young, and aggressive management is recommended.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Menopause is associated with a large number of symptoms ranging from physical to psychological. These symptoms may unfavorably affect oral health and treatment needs requiring dentists to be aware of the symptoms and health care needs of peri-menopausal/menopausal/postmenopausal women. This article attempts to provide an insight into the multifarious oral manifestations at menopause along with the relevant prosthodontic implications.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

True hermaphrodite is one of the rare variety of disorders of sex development. Most of them are genotypically females (46 XX) and present as under virilized males. Features of hyperandrogenism are present in those reared as females. The commonest histological variety of gonad found in them is ovotestis, which is at risk for malignancy. We report a girl with 46 XY true hermaphrodite without ovotestis presenting as primary amenorrhea and isolated clitoromegaly in the absence of any other features of virilization.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Thiamine responsive megaloblastic anemia syndrome, an autosomal recessive inherited disorder characterized by a triad of anemia, diabetes mellitus and sensorineural deafness is caused by a deficiency of a thiamine transporter protein. The disorder is rare and has not been reported from our community which has high background of consanguinity. We report a six years old girl who presented with diabetes mellitus which remitted after thiamine replacement. The girl in addition had sensorineural deafness, reinopathy, atrial septal defect and megaloblastic anemia which responded to high doses of thymine. This is the first case reported from Kashmir valley and third from India. The presentation and management in such cases is discussed.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

There is considerable evidence for the fact that early life environment in human beings are associated with future development of various metabolic diseases. Fetal programming and perinatal events appear to exert effects on later life that are independent of environmental risk factors in adults. Our understanding of the underlying mechanisms are limited and remains unclear. However several animal models and epidemiological studies have shown this association, and it is assumed secondary to the penalties of developmental plasticity. In this review, we amalgamate facts from several disciplines to support this hypothesis.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Turner's syndrome is a genetic disorder with a complete or partial absence of one X chromosome with characteristic phenotypic features. The prevalence of renal anomalies in turner syndrome is 30-40%. However, the renal function is usually normal. We report a case of Turner's syndrome presenting with chronic kidney disease and renal osteodystrophy.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]

McCune-Albright syndrome (MAS) is a sporadic disease characterized by polyostotic fibrous dysplasia, "café-au-lait" spots and hyperfunctional endocrinopathies. Pathophysiological basis is activating mutation of the gene that encodes the alpha subunit of Gs membrane protein that stimulates the intracellular production of cAMP, conferring autonomous secretion of the gland in particular. One of the uncommon endocrine manifestations is hyperthyroidism. We present a patient who had café-au-lait spots, polyostotic fibrous dysplasia and hyperthyroidism. She was treated with radioactive iodine for the symptoms of hyperthyroidism and subsequently relieved from hyperthyroid features.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Familial hypercholesterolema (FH) is an inherited autosomal dominant disorder of lipid metabolism. We report a 3 years old female child who presented with multiple eruptive xanthomatosis of skin since 6 months of age and had deranged lipid profile consistent with FH.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Introduction: Different growth and neuro-developmental outcomes have been associated with different doses of thyroxine given to infants with congenital hypothyroidism (CH). Materials and Methods: We studied the longitudinal growth pattern and assessed the neurodevelopment of 45 children with CH(25 girls, 20 boys) diagnosed through the national screening program in Qatar, for 6 years or more to examine the effects of initial T4 dosage (50 μg/day) with adjustment of T4 dose to maintain serum free T4 concentrations within the upper quartile of normal range and thyroid stimulating hormone < 4 mIU/mLThe birth size of newborns with CH diagnosed through the screening program before January 2003, was recorded and their growth in weight and stature was monitored every 3 months for at least 6 years of life. The IQ of children was assessed between 3 and 6 years of age using The Wechsler Preschool and Primary Scale of Intelligence (WPPSI-III). Results: Birth weight, length, and head circumference of patients (3.21 ± 0.43 kg, 50.5 ± 3.21 cm and 34.1 ± 1.5 cm, respectively) did not differ than those for 10,560 normal newborns with normal thyroid function (3.19 ± 0.59 kg, 50.5 ± 2.2 cm and 34.2 ± 1.7 cm). During the first year CH children growth (25.8 ± 2.8 cm/year) was similar to those for normal infants (25.5 ± 0.75 cm/year). During the first 6 years, stature growth was normal in all children with CH versus Center for disease control and prevention (CDC) data. The mean height standard deviation score (HtSDS) of children with CH showed adjustment (± 0.5 SD) toward their mid-parental height SDS (MPHtSDS) only during the second year of life. The children's mean HtSDS was higher by an average of 0.4 SD between the 2 ^nd and 7 ^th year of life. Conclusion: These data proved that effective screening and treatment completely assures normal neurodevelopment and linear growth in patients with CH. The data showed that their HtSDS slightly exceeds their MPHtSDS during childhood.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Fasting during the month of Ramadan, the ninth month of Islamic lunar calendar, is obligatory for all healthy adult and adolescent Muslims from the age of 12 years. Fasting starts from early dawn (Sohur/Sehri) till sunset (Iftar). During this period one has to abstain from eating and drinking. Islam has allowed many categories of people to be exempted from fasting, for example, young children, travelers, the sick, the elderly, pregnant, and lactating women. According to expert opinion, patients with type 1 diabetes (type 1 DM) who fast during Ramadan are at a very high risk to develop adverse events. However, some experienced physicians are of the opinion that fasting during Ramadan is safe for type 1 DM patients, including adolescents and older children, with good glycemic control who do regular self-monitoring and are under close professional supervision. The strategies to ensure safety of type 1 diabetic adolescents who are planning to fast include the following: Ramadan-focused medical education, pre-Ramadan medical assessment, following a healthy diet and physical activity pattern, modification in insulin regimen, and blood glucose monitoring as advised by the physician.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

A 5½-year-old adopted girl was referred to us in view of short stature. After ruling out systemic illness, she was evaluated for growth hormone deficiency (GHD) by stimulation tests. The peak value was 3.47 ng/ml. She was then started on growth hormone (GH). At the end of 6 months of GH therapy, her height velocity was only 3 cm/year. There was a lack of attachment between the mother and the child. She had history of hyperphagia, stealing, and hoarding food. Psychiatry consultation confirmed that the child had appetite disorder, and hence was diagnosed as hyperphagic short stature (HSS). The girl and her parents are undergoing psychiatric therapy for the same. Psychosocial dwarfism seems to originate from serious disturbances in the mother-child relationship. These children mimic patients with GHD, but have poor response to GH therapy. This case underscores the importance of social environment in the growth of the individual.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Ramadan is a lunar based month, during which Muslims across the world observe the ritual fast. This provides a challenge not only to the diabetic patient who wishes to observe the fast but also to the health care professional managing his diabetes. The challenge is to use therapies which are effective in maintaining good glycemic control and at the same time have a low propensity to cause hypoglycemia during the several hours of no calorie intake. The GLP-1 analogues are unique agents which are effective in providing glycemic reduction with a very low risk of hypoglycemia and hence find an important place in the management of diabetes during Ramadan. This Consensus Statement describes the pre-Ramadan assessment, planning, prescription and management and monitoring of patients who are on GLP-1 analogues, with or without other antidiabetic therapies.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Background: Postprandial hyperglycemia has been shown to be an independent cardiovascular risk factor. Some studies have reported that postprandial hyperglycemia is common and can occur even in patients with normal fasting glucose levels. This has been referred to as isolated postprandial hyperglycemia. Objectives: This study sets out to estimate the prevalence of isolated postprandial hyperglycemia in a cohort of patients with type 2 diabetes and to identify their clinical characteristics. Materials and Methods: Ninety patients being managed for type 2 diabetes were recruited consecutively as they attended the diabetes clinic for follow-up. The patients were assessed with questionnaires, to obtain the demographic data. Their body mass index (BMI) was calculated. Fasting blood samples were collected for analysis of fasting plasma glucose (FPG). Patients were given their usual drugs and then served a standard meal calculated to contain 50 g of carbohydrate, providing 500 kcal. Blood samples were collected two hours after the start of the meal for postprandial glucose levels. Results: The mean age of the patients was 57.7 ± 10.8 years with a male : female ratio of 2 : 3. The mean duration of diabetes was 6.77 ± 6.53 years. The mean BMI was 27.54 ± 6.01 kg / m ² . The mean FPG and two hour postprandial glucose were 7.51 ± 3.39 mmol / l and 11.02 ± 4.03 mmol / L, respectively, and the mean HBA1c was 9.0 ± 2.5%. The prevalence of isolated postprandial hyperglycemia was 24.4%. Elevated postprandial glucose was seen in 41.7% of the patients at target glycated hemoglobin levels. The patients with isolated postprandial hyperglycemia tended to be older and less obese. Conclusion: There was poor glycemic control in the patients generally; however, a significant proportion of patients, with apparently good glycemic control, had isolated postprandial hyperglycemia.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]

Thyroid nodules are very common in the general population, but only 5% are malignant. We report the case of a man presenting with nodular goiter, and multiple swellings over the scalp and arm, with pathological fractures. On evaluation by fine needle aspiration, these swellings were found to be metastases from follicular thyroid carcinoma. The patient underwent total thyroidectomy, radioiodine ablation, resection of the bone lesion, and L-thyroxine suppressive therapy, with no recurrence / metastasis over a five-year follow-up.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Occurrence of second tumors has been seen in patients with papillary and follicular thyroid cancer.We studied the occurrence of meningioma as a second neoplasm in patients with differentiated thyroid cancer treated with radioiodine at our institution.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

Background; Hyperglycemia plays an important role in etiology of vascular complications like atherosclerosis in diabetes. Oxidative modification of low density lipoprotein (LDL) plays an important role in the progression of atherosclerosis. The aim of the present study is to compare the antioxidant potential (AOP) of LDL in type 2 diabetic men and women. Materials and Methods: The study was carried out in 80 diabetic subjects and 80 control subjects. The men (40) and women (40) in the diabetic groups were studied separately and matched for age (50-60 years), body mass index (BMI), duration of diabetes, glycosylated hemoglobin, and lipid profile. LDL from the serum sample was precipitated by heparin citrate precipitation method. For the measurement of AOP in LDL, we used xanthine-xanthine oxidase method. Results: Our results showed that AOP value was significantly low in diabetic women (P < 0.05) in comparison with diabetic men. Conclusion: It is therefore suggested that LDL from type 2 diabetic women is more prone for oxidation.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]

Objectives: There are few studies reporting lipid profile in cystic fibrosis (CF) and most of them are in adult Caucasians. Therefore, the aim of this study was to evaluate the lipid profile of racially admixtured youths with CF. Materials and Methods: A cross-sectional survey conducted between August and September 2009 at a reference service for CF, evaluating clinical and laboratory data. Results: Forty-six patients aged from 6 years to 16 years and 2 months (median: 9 years and 10 months; 65.2% males) were evaluated. Of these, 26% were Whites, 54.4% Mulattoes and 19.6% Blacks. There were no diabetics, one patient had glucose intolerance and three had insulin resistance. Pancreatic sufficiency was present in 74% and malnutrition in 26% of the patients. The lipid profile revealed hypertriglyceridemia in 56%, hypercholesterolemia in 17.4% and hypocholesterolemia in 46.5%. In 30.4% of the patients, hypertriglyceridemia and hypocholesterolemia was observed. The serum levels of high density lipoprotein (HDL) were low in 56.5% and the low density lipoprotein (LDL) elevated in 15.2% of the patients. Conclusions: The lipid profile of this sample of Brazilian racially admixtured patients with CF showed a higher prevalence of hypertriglyceridemia and hypocholesterolemia. There was no association of dyslipidemia with the various racial groups, nutritional status, pancreatic sufficiency or glucose tolerance.

[ABSTRACT]  [FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub]

[FULL TEXT]  [PDF]  [Mobile Full text]  [EPub] [CITATIONS]