39 research outputs found

    Is it T3 thyrotoxicosis? A case of falsely elevated tri-iodothyronine (T3) levels leading to a diagnosis of multiple myeloma

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    We are presenting a case of falsely elevated T3 levels in a patient due to interference from monoclonal immunoglobulins. A 56-year-old, clinically euthyroid man referred to the endocrinology clinic of the Aga Khan university, Karachi Pakistan, for possible T3 thyrotoxicosis after thyroid function tests revealed total T3 \u3e12.32 nmol/L (reference range 0.6-2.79), normal TSH, and total T4 level. There was a mismatch in clinical and laboratory parameters and preliminary laboratory results were suggestive of thyroid binding globulin abnormalities. Further evaluation in this context unmasked multiple myeloma. The presence of monoclonal immunoglobulins can lead to assay interference and spurious results. To the best of our knowledge, this is the second case defining the cause of falsely elevated T3 levels, due to assay interferences with binding of T3 only to monoclonal immunoglobulins

    A case report: Familial glucocorticoid deficiency associated with familial focal segmental glomerulosclerosis

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    Background: Familial glucocorticoid deficiency (FGD) is a rare autosomal recessive disorder characterized by isolated glucocorticoid deficiency in the presence of normal plasma renin and aldosterone level. Focal segmental glomerulosclerosis (FSGS) is a form of glomerular disease associated with proteinuria and nephritic syndrome. This is the first case of familial glucocorticoid deficiency associated with familial focal segmental glomerulosclerosis. Case Presentation: An eight month old boy presented with increased genital pigmentation. Initial investigation revealed that he was glucocorticoid deficient and was started on hydrocortisone and fludrocortisone with a diagnosis of primary adrenal insufficiency. Later fludrocortisone was withdrawn and he was diagnosed to have isolated glucocorticoid deficiency. He later developed focal segmental glomerulosclerosis for which he underwent renal transplantation at the age of five years. Now at the age of twelve years, this boy is doing well on hydrocortisone treatment. His two siblings and a first degree cousin also had isolated glucocorticoid deficiency. One of the above two siblings died due to renal failure secondary to focal segmental glomerulosclerosis. Conclusion: Patients with familial glucocorticoid deficiency should be carefully followed for development of features of nephrotic syndrome

    Patterns of abnormal glucose metabolism in acromegaly and impact of treatment modalities on glucose metabolism

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    Background: Chronic exposure to high levels of growth hormone (GH) and insulin-like growth factor 1 (IGF-1) leads to metabolic complications, most importantly dysglycemia in the form of diabetes mellitus and pre-diabetes. Dysglycemia if diagnosed early in the course of the disease can decrease complications. Treatment modalities in the form of surgery and medical therapy have varied impacts on glucose metabolism.Objective: To determine the frequency of diabetes mellitus, impaired glucose tolerance, and impaired fasting glucose in Pakistani patients with acromegaly and to establish the impact of the intervention (surgery/medical) on glucose metabolism.Methods: This study was a retrospective review of patient records. Eighty-nine patients fulfilling the Endocrine Society criteria for acromegaly diagnosis were included. A data of baseline, GH, IGF-1 level, hemoglobin A1C (HbA1C), fasting blood glucose (FBG), and random blood glucose (RBS) levels were reviewed before and after the intervention (surgery/medical therapy). Normal glucose tolerance (NGT), impaired fasting glucose (IFG), impaired glucose tolerance (IGT), and diabetes mellitus (DM) were defined based on the American Diabetes Association (ADA) criteria. Patients were grouped into normoglycemic (NGT) and dysglycemic (IFG, IGT, and DM) based on FBG, RBS, and HbA1C.Results: Major risk factors for dysglycemia included age (15-45 years), male sex (33.70%), obesity (45.7%), and macroadenoma (77.52%). Both mean GH levels (58.29 vs. 54.36 ng/dl) and IGF-1 levels (862.98 vs. 824.32 ng/dl) were higher among the normoglycemic than dysglycemia. Pre-surgery NGT, IFG, IGT, IFG, and IGT/DM combined were found in 48.31%, 5.61%, 1.1%, 5.61%, and 39.32% of the subjects respectively. Post-surgery, HbA1C improved in 79.5%, deteriorated in 6.8%, and remained the same in 13.6%. Similarly, it improved in 67% post-medical therapy. Both FBG and RBS improved post-surgery and medical therapy. Further, the number of anti-diabetic drugs used also decreased post-surgery.Conclusion: Dysglycemia is more common among patients with acromegaly as compared to the general population and tends to be poorly controlled in untreated acromegaly. Glycemic control improves significantly after surgery and medical therapy

    Male hypogonadism at a tertiary care hospital in Karachi, Pakistan

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    Background: Male hypogonadism is defined as \u27inadequate gonadal function, manifested by deficiency in gametogenesis and/or secretion of gonadal hormones\u27. Signs and symptoms of hypogonadism depend primarily on the age of onset. It can be classified according to the site primarily involved: the gonads, the hypothalamus, or the pituitary gland. The objective this study was to determine the presentation and aetiology of male hypogonadism seen in a tertiary care hospital. Methods: This cross-sectional study was conducted at Endocrine Clinics, Aga Khan University Hospital Karachi. Data of male patients with hypogonadism who attended clinics during January 2009 to August 2011 were reviewed. All male patients with clinical and biochemical evidence of hypogonadism were included in the study. Patients with Diabetes Mellitus, Metabolic Syndrome, Andropause, AIDS, Chronic Renal Failure, and Cirrhosis were excluded. Mean +/- SD were computed for quantitative variables. Frequency and percentages were computed for qualitative variables. Aetiology of male hypogonadism was categorised as primary and secondary hypogonadism. Results: A total of 85 patients with male hypogonadism attended the endocrine clinic. Mean age of patients was 25 +/- 10 years. Clinical presentations were small genitalia (65%), absent secondary sexual characteristics (53%), not attained puberty (47%), infertility (53%), erectile dysfunction (41%) and loss of libido (29%). Seventy-three (86%) patients had hypogonadotrophic hypogonadism (secondary hypogonadism) and 12 (14%) patients had hypergonadotrophic hypogonadism (primary hypogonadism). Among the patients with hypogonadotrophic hypogonadism 38 had idiopathic hypogonadotrophic hypogonadsim, 7 had pituitary adenoma, 6 had empty sella syndrome, 3 had Kallman\u27s syndrome, and 1 patient had haemosiderosis due to thalassaemia major; 18 patients did not undergo brain imaging. Conclusion: Small genitalia, absent secondary sexual characteristics and infertility were the main presenting features of hypogonad men. Majority of patients had hypogonadotrophic hypogonadsim

    Idiopathic hypogonadotropic hypogonadism reversal after testosterone replacement in a 34-year-old male

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    A 34-year-old male presented to the endocrinology clinic with the complaint of the absence of facial, axillary and pubic hairs. Further history revealed absent ejaculations and decreased early morning erections. The patient had no history of headaches, visual problems or anosmia. On physical examination, there were sparse facial, axillary and pubic hairs, bilateral gynaecomastia, stretch penile length of 5 cm and bilateral testicular volume of 10 mL. Laboratory investigations showed low luteinising hormone, follicular stimulating hormone and testosterone with normal prolactin and thyroid profile. MRI of the pituitary gland showed no evidence of pituitary microadenoma or macroadenoma. The patient was started on testosterone injections. After 9 months of testosterone replacement, the patient\u27s testicular size increased to 20 mL bilaterally and his penile length increased to the mean adult size for his age with normal testosterone and luteinising hormone. He was, thus, advised to discontinue testosterone therapy

    Importance of measuring non-HDL cholesterol in type 2 diabetes patients.

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    OBJECTIVE: To study the correlation between Non-high-density lipoprotein and low-density lipoprotein cholesterol in patients with Type 2diabetes mellitus and the proportion of patients achieving Adult Treatment Panel III recommended goals. METHODS: The cross sectional study was conducted at the Diabetic Clinic, Aga Khan University Hospital, Karachi. Data of Type 2 diabetesmellitus patients who attended the clinic bewteen 2007 and 2011 was reviewed. All Type 2 diabetic patients of either gender with fasting lipid profile irrespective of taking lipid lowering therapy were included.Type-1 DM, gestational diabetes, type 2 diabetes patients with pregnancy and those with incomplete data were excluded. Correlation between the low-density lipoprotein and Non-high-density lipoprotein was assessed by applying Cramer V and phi. Proportion of patients achieving Adult Treatment Panel III recommended goals was checked. Multivariable regression was done to identify common factors associated with elevated Non-high-density lipoprotein cholesterol. RESULTS: A total of 1352 patients fulfilling the eligibility criteria were included in the study. Mean age of the patients was 54.5 +/- 11.3 years; 797 (59%) were males; 1122 (83%) had Body Mass Index above 25; and 1016 (75%) had HbA1c \u3e or = 7%. Mean Non-high-density lipoprotein cholesterol was 129 +/- 42 mg/dl. Mean low-density lipoprotein cholesterol was 100 +/- 37 mg/dl. Both low-density lipoprotein \u3c or = 100 and Non-HDL \u3c or = 130 mg/dl was achieved in 645 (48%) patients. It is important to note that although 728 (53.8%) patients achieved target LDL cholesterol of \u3c or = 100 mg/dl, among them 83 (11.4%) had Non-high-density lipoprotein cholesterol still above the target \u3e 130 mg/dl (p \u3c 0.05). Out of 752 patients with Non-high-density lipoprotein cholesterol \u3c or = 130 mg/dl, 645 (86%) had low-density lipoprotein cholesterol below 100 mg/dl. Cramer V and Phi showed that correlation between Non-high-density lipoprotein and low-density lipoprotein cholesterol was 0.71 (p value \u3c 0.01). After adjusting for other covariates, low-density lipoprotein cholesterol \u3e 100 mg/dl was independently associated with having Non-high-density lipoprotein cholesterol \u3e 130 mg/dl (Adjusted Odds Ratio 38.6; 95% Confidance Interval = 28.1-53.1). Similarly, age \u3c or = 60 years was 60% more likely to have Non-high-density lipoprotein cholesterol \u3e 130 mg/dl (Adjusted Odds Ratio 1.6; 95% Confidance Interval = 1.01 - 2.3). Whereas having obesity Body Mass Index \u3e 25 was 3.6 times more associated to have Non-high-density lipoprotein \u3e 130 mg/dl (Adjusted Odds Ratio 3.6; 95% Confidance Interval = 1.6-7.7). In patients with coronary artery disease, combined goal achievement of low-density lipoprotein \u3c or = 70 mg/dl and Non-high-density lipoprotein cholesterol \u3c or = 100 mg/dl was seen in 59 (35%). Among patients with high-density lipoprotein \u3c or = 70 mg/dl, 8 (10%) had Non-high-density lipoprotein \u3e 100 mg/dl (p \u3c 0.05). CONCLUSION: The study showed a correlation between Non-high-density lipoprotein and low-density lipoprotein cholesterol. As measuringNon-high-density lipoprotein cholesterol in Type 2 DM patients is simple, cost-effective and convenient because it does not require 12-hour fasting which may be a risk for hypoglycaemia in these patients, clinicians may choose Non-high-density lipoprotein as a routine measure in everyday practice

    Pituitary stalk interruption syndrome presenting as short stature: a case report

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    Introduction: Pituitary stalk interruption syndrome is a rare congenital abnormality of the pituitary that is responsible for anterior pituitary deficiency. It is characterized by a classic triad of interrupted pituitary stalk, absent or ectopic posterior pituitary, and anterior pituitary hypoplasia or aplasia. Clinical presentation varies according to age. In adults it presents as short stature and anterior pituitary deficiency. Without early diagnosis and treatment, mortality and morbidity in these patients is high. Early diagnosis and treatment of this rare disease can prevent permanent short statue of the patient. We report the first case of pituitary stalk interruption syndrome from Pakistan. CASE PRESENTATION: A 17-year-old Pakistani young man presented with short stature and underdeveloped secondary sexual characters. His siblings and parents were healthy, with normal height. An examination showed his blood pressure was 90/60 mmHg, and his height, weight, and body mass index were 142 cm, 34.5 kg, and 17.10 kg/m2, respectively. He had no hair growth on his face, axilla, or pubis. His testes were between 1 and 2 mL in size, with a 4 cm-at-stretch micropenis. His lab investigations showed that his thyroid stimulating hormone (TSH) was 8.58 uIU/mL (0.4 to 4.2), his free thyroid hormone level FT4 was 0.46 ng/dL (0.89 to 1.76), his prolactin was 21.1 ng/mL (3.0 to 14.7), and his baseline cortisol was 0.30 ug/dL (4.3 to 22.4). His cortisol level after 60 minutes of cosyntropin injection was 3.5 ug/dL (4.3 to 22.4), his insulin like growth factor IGF-1 was 31.56 ng/mL (247.3 to 481.7), his testosterone level was under 2.5 ng/dL (2 to 800), his follicle stimulating hormone FSH was 0.41 uIU/mL (0.0 to 10.0), and his leutinizing hormone LH was under 0.1 uIU/mL (1.2 to 7.8). His bone age was 10 years according to the Greulich and Pyle method, as shown by X-rays. The results from his pituitary magnetic resonance imaging scan were consistent with pituitary stalk interruption syndrome. CONCLUSIONS: We describe a young man who presented with short stature and was found to have pituitary stalk interruption syndrome. Despite the fact that this is a rare disorder, it should always be kept in the differential diagnosis of a patient presenting with short stature. Patients with this disease have an excellent opportunity to reach normal height if they present before the joining of epiphyses

    An Analysis of Rural Poverty Trends in Sindh Province of Pakistan

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    Abstract: The economy of rural Sindh depends upon agriculture sector. About 65% populations are engaged in this sector. Irrigation is the main component for the agriculture sector. Therefore, Sindh is dependent on the Indus Basis Irrigation System. Any shortage in the requirement of irrigation for Sindh Province will affect the rural economy. The increasing trends of poverty were observed during 1990's in rural Sindh due to shortage of irrigation water. The allocated share of water was 33.51 MAF for Kharif and 13.23 M AF for Rabi seasons. Besides, 10 MAF was allowed to down stream Kotri for the survival of Indus Delta. The outflow down stream Kotri continuously decreased to 0.28 MAF during 2008-09 which affected Indus Delta as well as about 1.5 million acres fertile agricultural land of districts Thatta and Badin came under sea water. The 3.5 lac people of both districts shifted in other areas of the country in search of their livelihood. In addition to, the other factors like increasing land degradation, water logging and salinity, deforestation, soil erosion, uneconomic holding and drought were responsible for increasing trends of poverty in Sindh province

    Design and performance analysis of PV grid-tied system with energy storage system

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    With the increasing demand for solar energy as a renewable source has brought up new challenges in the field of energy. However, one of the main advantages of photovoltaic (PV) power generation technology is that it can be directly connected to the grid power generation system and meet the demand of increasing energy consumption. Large-scale PV grid-connected power generation system put forward new challenges on the stability and control of the power grid and the grid-tied photovoltaic system with an energy storage system. To overcome these problems, the PV grid-tied system consisted of 8 kW PV array with energy storage system is designed, and in this system, the battery components can be coupled with the power grid by AC or DC mode. In addition, the feasibility and flexibility of the maximum power point tracking (MPPT) charge controller are verified through the dynamic model built in the residential solar PV system. Through the feasibility verification of the model control mode and the strategy control, the grid-connected PV system combined with reserve battery storage can effectively improve the stability of the system and reduce the cost of power generation. To analyze the performance of the grid-tied system, some real-time simulations are performed with the help of the system advisor model (SAM) that ensures the satisfactory working of the designed PV grid-tied System
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