Abstract: STUDY OBJECTIVE: To study the clinical, metabolic and adverse effects of pioglitazone over a period of 6 months in obese adolescent and young adults with polycystic ovary syndrome. DESIGN: This was an open labeled study. Each patient served as her own control. SETTING: Outpatient department of a university affiliated teaching hospital. PARTICIPANTS: Unmarried women (age 15-25 yrs) with chronic anovulatory cycles and obesity, and with clinical evidence of hyperandrogenism. INTERVENTIONS: Pioglitazone at a dose of 30 mg once daily for a period of 6 months along with dietary advice and exercise. MAIN OUTCOME MEASURES: Resumption of normal menstrual cycles, clinical improvement in hyperandrogenism and changes in insulin resistance measured by fasting glucose insulin ratios. RESULTS: Twenty-two women were enrolled. At the end of the study period 91% of the subjects had regularization of menstrual cycles. There was no change in the modified Ferriman-Gallwey hirsutism scores. Decline in fasting insulin levels at the end of the study was 45.6% from baseline along with significant increase in the fasting glucose/insulin ratio from baseline. CONCLUSION: Administration of pioglitazone for 6 months along with advice about diet and physical activity in obese adolescents and young adult women with polycystic ovary syndrome results in significant improvements in menstrual frequency. There is a significant improvement in insulin resistance using the G/I ratio (<7.5 mg/10(-4) U) as the biochemical marker.

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Abstract: INTRODUCTION: Symptomatic skeletal disease in primary hyperparathyroidism is over 30 times more common in India compared to the west. The classical "brown tumour" is commonly seen with the major sites being ends of long bones, the pelvis and ribs. Facial involvement is rare and, when present, usually involves the mandible. CASE REPORT: We report a 68-year-old gentleman with a rare initial presentation of primary hyperparathyroidism with bilateral maxillary brown tumours. DISCUSSION AND CONCLUSION: Successful parathyroid surgery resulted in a regression in the tumours. The report highlights the need to consider primary hyperparathyroidism in the initial differential diagnosis of bony lesions of the jaw.

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Abstract: BACKGROUND AND AIM: Fluoroquinolones are antimicrobial agents with a broad spectrum of activity against gram-positive, gram-negative, and anaerobic organisms. They are widely used in surgical practice and are generally considered safe. Hypoglycemia because of use of levofloxacin is a rarely reported complication. This report of a case of a surgical patient highlights this potentially fatal complication. PATIENT: An elderly, non-diabetic patient with renal impairment presented with a possible duodenal perforation. After successful surgery, the patient developed recurrent hypoglycemic episodes in the post-operative period after use of levofloxacin. Delay in recognition of the cause of hypoglycemia led to irreversible brain damage and death. RESULTS: The calculated Naranjo adverse drug reaction probability scale criteria suggest the possibility that these episodes were related to levofloxacin. The mechanism of hypoglycemia with levofloxacin relates to the potential inhibition of the K(ATP) channel on the pancreatic beta cell by the drug. CONCLUSION: The case report highlights the need to be aware of this potentially fatal complication of a drug commonly used in surgical practice.

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Abstract: The aim of this study was to describe the localization and management of patients with pancreatic insulinomas and determine the most effective localization and surgical techniques in the presence of significant financial constraints in the patient population. We retrospectively reviewed the case records of 18 patients with insulinomas treated at our institution over a period of 10 years. The medical records were reviewed for demographic data, clinical presentation, biochemistry, details of localization studies, intraoperative findings, postoperative outcome, and long-term follow-up. The sensitivities of the various localization procedures were calculated using the intraoperative findings as the gold standard. There were 10 men and 8 women in the study, with a median age of 43 years. All patients underwent a supervised 72-hour fast and developed symptomatic hypoglycemia within 48 hours. An average of 1.9 localization procedures was performed per patient. Computed tomography (CT) had a sensitivity of 62% and specificity of 100%. Magnetic resonance imaging and digital subtraction angiography had specificities of 85% and 100%, respectively, with a specificity of 66% and 50%, respectively. Fourteen patients underwent surgery. Intraoperatively the excised tumor was palpable in nine patients, and all patients had postoperative euglycemia. In five patients the tumor was not palpable during the time of surgery; three of these patients underwent blind distal pancreactomy, with two patients having persistent hypoglycemia during the postoperative period. Two patients had a negative exploratory laparotomy. Patients with a surgical cure were followed up for a mean period of 24 months. On the background of financial constraints in connection with patient care, CT scanning is a cost-effective option with good specificity. Intraoperative palpation of the tumor and enucleation is the most effective technique for surgical cure. Blind distal pancreactomy is not advocated for tumors that are not localized intraoperatively.

Abstract: A five-year-old boy presented with progressive weight gain with effort intolerance and nocturnal symptoms suggesting obstructive sleep apnoea. A clinical diagnosis of Prader-Willi Syndrome was made. As the initial radiography and computed tomography suggested a foreign body, bronchoscopy was done under general anaesthesia and impacted peanuts were removed from the left main bronchus. His symptoms resolved instantly and the patient was asymptomatic at six months follow-up. This report highlights the need to consider foreign body aspiration as a cause for dyspnoea in children with Prader-Willi Syndrome. The report also focuses on the need to adopt strategies that prevent foreign body aspiration and choking in patients with Prader- Willi Syndrome.

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Abstract: A 38 year old gentleman presented with redness of both eyes of 15 days duration associated with severe bifrontal, non-throbbing headache. Ophthalmological evaluation revealed bilateral scleritis and retinal haemorrhage in the supero-temporal quadrant. He also had hyperuricemia and was referred to our institution with the suspicion of a haematological malignancy. Biochemical investigations done by us showed presence of hypercalcemia and hypophosphatemia, with elevated alkaline phosphatase and parathyroid hormone (PTH) levels, suggesting a diagnosis of primary hyperparathyroidism. This was confirmed when ultrasound imaging of the neck and the Technetium-Sestamibi scanning revealed a left inferior parathyroid adenoma. Following excision of the adenoma, the redness of the eyes resolved over 2 months. For primary hyperparathyroidism to initially present with red eyes is quite unusual.

Abstract: A 4-year-old boy presented with delay in developmental milestones and aggressive behavior. An axial CT scan of the brain was done. Subsequent evaluation revealed normal serum calcium and inorganic phosphorus values with an elevated serum alkaline phosphatase. His arterial blood gas analysis was suggestive of metabolic acidosis with anionic gap of minus 15.1. His skeletal X-rays were done.

Abstract: BACKGROUND: Familial pure gonadal dysgenesis with 46 XX karyotype and sensorineural deafness constitutes a rare autosomal recessive syndrome described initially by Perrault in 1951. The spectrum of the disease remains undetermined. Families with additional newer findings are regularly reported. CASE: We report two siblings with gonadal dysgenesis, progressive sensorineural deafness, Marfanoid body proportions and skeletal features, and a normal female karyotype. The diagnosis of Perrault syndrome was made. Abnormal body proportions including a longer arm span, shorter trunk, high arched palate, long slender fingers and positive thumb and wrist sign were observed. The siblings did not have any cardiac or ocular features of Marfan's syndrome. CONCLUSION: The report of the siblings adds to the expanding spectrum of findings in Perrault syndrome.

Abstract: The widespread use of Magnetic Resonance Imaging (MRI) has resulted in the discovery of unsuspected endocrinologically silent masses in the pituitary gland (pituitary incidentalomas). Pituitary enlargement in patients with long-standing primary hypothyroidism is well established and is reported to correlate with circulating thyrotropin levels. We report two cases where the discovery of pituitary masses led to the diagnosis of primary hypothyroidism that was not suspected previously. This report highlights the importance of early evaluation of thyroid functions in patients with incidentally discovered pituitary masses and avoiding expensive referrals.

Abstract: OBJECTIVE: To report the case of a woman with idiopathic sporadic tumoral calcinosis treated successfully with orally administered bisphosphonates. METHODS: We report the clinical, laboratory, and imaging findings and describe the clinical course of tumoral calcinosis. The literature was reviewed for the pathophysiologic features and strategies for treatment of tumoral calcinosis. In addition, we specifically reviewed the use of bisphosphonates in tumoral calcinosis and the possible pharmacologic basis for the therapeutic benefit. RESULTS: A 45-year-old woman presented with a 6-week history of left-sided pain in the hip in conjunction with stiffness after a trivial fall 4 weeks before the onset of symptoms. The findings on conventional radiology of the hip joint were consistent with tumoral calcinosis of the left hip. The biochemical profile of the patient was unremarkable. Oral treatment with alendronate, 70 mg once a week, yielded alleviation of symptoms within 12 weeks. Radiology of the left hip repeated after a period of 15 months revealed notable regression of the calcified lesion. CONCLUSION: Bisphosphonate therapy may be considered as an alternative to surgical treatment in patients with idiopathic sporadic tumoral calcinosis.

Abstract: A 5 year old boy was brought to us because his parents had noticed a rapid increase in his height over the last 2 years with deepening of his voice in the last one year and premature maturation of his genitalia in the last 6 months. On examination he was 137cm tall, which was much above the 97th percentile for his age with a height SD Score of +2.5 SD. He had well developed muscles and weighed 30kgs. On genital examination he had bilateral testicular volumes of 10ml, pubic hair of Tanner Stage 3 and adult sized penis. There were no skin lesions or skeletal abnormalities. The fundus and neurological examination were normal. On laboratory examination he had serum total testosterone level and serum gonadotropin levels in the normal adult range. Blood levels of 17-hydroxy Progesterone, DHEAS and cortisol were within the normal limits. The bone age was estimated to be around 12.5 years. An MR imaging of the pituitary region revealed a normal sella and pituitary gland. There was a well defined homogenous lesion in the basal cistern below the mammilary body of 14x14x11mm dimensions which was iso-intense to the gray matter on T1 weighed images and did not enhance with Gadolinium.

Abstract: A five and half year old girl child presented to us with history of onset of menarche at the age of 4 years with bilateral lower limb pain over twelve months. Historically she had been born to non-consanguineous parents at term. She had no previous medical illnesses and no prior history of any medications. Physical examination revealed a normal statured (height standard deviation score of plus 1.3 based on western data) normotensive girl, with a large pigmented skin lesion in the right T4-T5 dermatome (Figure 1). She had a genu valgum with inter malleolar distance of 3 cms and no other bony swellings or deformities. There was evidence of early thelarche (Breast; Tanner Stage 2) and no evidence of adrenarche. Systemic examination was normal. Results of laboratory tests (and normal ranges shown in parenthetically) were as follows: Serum Estradiol <0.07nmol/L (Women luteal phase 0.1-0.91 nmol/L), Serum LH <0.10mIU/ml (Women luteal phase 0.0 – 14.7mIU/ml), Serum FSH 0.81mIU/ml (Women luteal phase 1.2-9.0 mIU/ml), Serum TSH – 1.47mIU/ml (0.3 - 4.5mIU/ml). Serum Calcium, phosphorus, liver and renal functions were normal. X ray of the wrist (Figure 2) showed cystic lesions in the metacarpal bones with advancement of the bone age by three and a half years. X-ray of the tibia also revealed an early expansile cystic lesion (Figure 3). What is your diagnosis?

Abstract: A 59-year-old gentleman presented with symptoms of progressively worsening low back pain associated with difficulty in rising from a squat over a period of two years. Biochemical tests confirmed the initial clinical diagnosis of osteomalacia. Blood pool scanning revealed a focal hot spot on the site of the clinically visible swelling close to the metacarpo-phalangeal joint of the left index finger. The biopsy of the specimen obtained by excision was reported to be consistent with a phosphaturic mesenchymal tumour. The patient had complete resolution of symptoms six months following excision of the lesion.

Abstract: The association of monoclonal gammopathies with primary hyperparathyroidism is well documented. Many case reports have documented the coexistence of primary hyperparathyroidism and multiple myeloma. The cause of this relationship is not known. We report the case of a 49-year-old gentleman who was treated for primary hyperparathyroidism. His initial preoperative nuclear scan had shown persistent activity and retention of tracer in the retrosternal region in addition to the discrete hot spot in the region of the lower pole of the left lobe of the thyroid. During surgery, the enlarged left inferior parathyroid gland was removed. In addition, the retrosternal area was also explored and found to be normal. Ten months later, he developed a mass in the region of the manubrium sternii which was proven to be a plasmacytoma. Were view the literature for similar cases and suggest hypotheses for a possible association. In conclusion, coexisting plasma cell dyscrasias including plasmacytoma should be considered in patients with primary hyperparathyroidism.

Abstract: Intracardiac blood cysts are thin-walled congenital cysts located in the endocardium and are seen predominantly in infants. They are rare in adults and are typically asymptomatic. However, complications such as valve dysfunction and left ventricular outflow tract obstruction have been reported in adults. We report a 42-year-old woman who developed an embolic stroke in association with intracardiac blood cysts. To the best of our knowledge, this is the first case report of intracardiac blood cysts manifesting as an embolic stroke.

Abstract: Vomiting as a predominant presenting symptom is unusual in thyrotoxicosis. Various mechanisms have been proposed for this presentation. We present the case record of a 62 year old lady with thyrotoxicosis and recurrent vomiting. During the course of her evaluation we uncovered multiple reasons for her presenting symptoms.

Abstract: Tumoral calcinosis is a rare calcifying disorder that is associated with deposition of calcium crystals in the periarticular tissues. The mass is most often around the hips, elbows, shoulders, and feet but may be occasionally found elsewhere. We report a case of multiple sporadic tumoral calcinoses in an adult male over the scalp. The scalp as a site of tumoral calcinosis has not been previously reported in adults. Previous surgical excisions done on two occasions had resulted in recurrence of the tumors. This report highlights the need to include tumoral calcinosis in the differential diagnosis of tumors of the scalp.

Abstract: BACKGROUND: Management of medullary thyroid carcinoma (MTC) remains controversial despite many advances over the past five decades. We attempt to review the presentation, management and prognosis of MTC at our institution over the last two decades. METHODS: We conducted a retrospective review of the records of 40 patients with MTC over a period of 20 years. RESULTS: Ten patients had hereditary MTC and 30 had sporadic MTC. The mean age of presentation was 41 years. Sixty-five per cent of the patients had a definite thyroid swelling and 43% had lymphadenopathy at the time of presentation. Total thyroidectomy with a central neck dissection was carried out in 82.5% of patients. Adjuvant therapy was given in 75% of patients because of extensive/residual disease. Postoperative hypercalcitoninaemia was seen 73% of patients. (131)I metaiodobenzylguanidine scanning was carried out in 16 patients with persistent hypercalcitoninaemia; the uptake was positive in 10 and negative in 6, indicating a positivity of 62%. CONCLUSION: Medullary thyroid carcinoma accounts for 2.5% of thyroid carcinomas. There is a small male preponderance. In our series (131)I metaiodobenzylguanidine scan had a better positivity than what has been reported in the published work. Persistent postoperative hypercalcitoninaemia was associated with a poorer prognosis that did not reach statistical significant.

Abstract: A 35-year-old male presented with right loin pain and two episodes of painless haematuria. At the age of 27,the patient had surgery for a left cerebellar astrocytoma followed by 6 weeks of radiation therapy. He had been asymptomatic for the last 8 years. On examination he was found to have hypertension and a palpable mass in the right lumbar region. Computed tomographic scans of the abdomen were performed to evaluate the mass (Figs a and b). What are the marked lesions and what is the diagnosis?

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Abstract: Papillary and follicular (differentiated) carcinomas of the thyroid are among the most curable cancers and together with medullary carcinoma and anaplastic carcinoma of the thyroid account for around 1% of all human cancers.

Abstract: BACKGROUND: The association between pancreatic disease and primary hyperparathyroidism (PHPT) is controversial. We attempt to suggest a causal correlation and characterize the nature of pancreatic disease in PHPT. METHODS: This study is a retrospective review of the medical records of patients who were diagnosed with PHPT and presented with pancreatic disease between 1 May 2000 and 30 May 2005 at Christian Medical College, Vellore. RESULTS: During the period of 5 years, there were 1284 patients with pancreatic disease and 101 patients with PHPT admitted to our hospital, accounting for 0.42 and 0.03%, respectively, of the total hospital inpatient admissions of 302 883. Of them, 13 patients had both pancreatic disease and PHPT accounting for 1% of all admissions for pancreatic disease and 12% of admissions for PHPT. Patients admitted with PHPT have a 28-fold increased risk of developing pancreatitis compared with patients admitted without parathyroid disease. The ages of the patients ranged from 22 to 52 years with a median age of 37 years. There were 9 male and 4 female patients. The mean calcium values among patients with PHPT and pancreatic disease were significantly higher than patients with PHPT without pancreatic involvement. CONCLUSION: The data suggest a causal association between the pancreatic disease and PHPT. This may be correlated to the higher calcium values. Until more information is available, it would be prudent to check serum calcium in all patients presenting with unexplained pancreatic disease.

Abstract: A 17 year old boy presented with history of fracture of the left humerus sustained on minimal trauma (Figure 1). There was previous history of multiple episodes of pathological fractures with deformities since childhood. Radiological survey of the patient revealed multiple expansile lesions in various long bones. (Figure 2 & 3). Further physical examination revealed a dark brown hyperpigmented lesion over the upper back with irregular borders. (Figure 4).

Abstract: Aims: To calculate the sensitivity, specificity and positive predictive value of Parathyroid scintigraphy using Technetium-Sestamibi scan in the localization of parathyroid disease. Methods and material: This is a retrospective medical review of 161 patients who underwent the Parathyroid scintigraphy at our hospital in last six years. True positives were defined as patients with confirmed primary hyperparathyroidism (PHPT) on pathological examination. True negatives were patients with negative biochemical evaluation and normal Parathyroid scintigraphy. False positives were patients with positive scans and no biochemical features of PHPT. False negatives were patients with a negative scan and biochemical hyperparathyroidism with a histological diagnosis of PHPT. Results: The sensitivity of Parathyroid scintigraphy (PS) for precise localization of parathyroid adenoma is 76% with a specificity of 78%. The positive predictive value for Parathyroid scintigraphy (PS) for accurate and unambiguous localization is 84.2%. Conclusions: 99mTc-Sestamibi remains a valuable tool for localization of preoperative parathyroid disease. In the present clinical setting it may not be sensitive enough to replace bilateral neck exploration with more limited surgical procedures.

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Abstract: Diabetic ketoacidosis (DKA) is a state of absolute or relative insulin deficiency aggravated by ensuing hyperglycemia, dehydration, and acidosis-producing derangements in intermediary metabolism. The most common causes are underlying infection, disruption of insulin treatment, and new onset of diabetes. DKA is typically characterized by hyperglycemia over 300 mg/dL, low bicarbonate (<15 mEq/L), and acidosis (pH <7.30) with ketonemia and ketonuria. Hyperosmolar hyperglycemic nonketotic coma (HHNC) is a metabolic derangement that occurs principally in patients with adult-onset diabetes. The condition is characterized by hyperglycemia, hyperosmolarity, and an absence of significant ketosis

Abstract: Hypoglycaemia is defined by a serum glucose level of less than 50 mg/dL. The glucose level at which an individual becomes symptomatic is highly variable. A delay in treatment can result in profound sequlae. Treatment should not be withheld while waiting for a laboratory glucose value. The brain uses glucose as its primary energy source hence neuronal damage may occur if treatment of hypoglycaemia is delayed.

Abstract: The adoption of a sedentary lifestyle is a clear factor in the development of Type 2 Diabetes Mellitus. The accumulation of the excess energy stores into the muscle cells and liver as free fatty acids and glycogen results in Insulin resistance. The clinician can increase the chances of the patient "buying into" the exercise regimen by asking the patient to set his own exercise goals. Although it would be ideal to have the individual with diabetes exercise daily, it is important to remember that even some exercise is better than none

Abstract: Objective: To study the clinical outcomes in terms of thyroid functions and overall cure rates at 3 and 6 months after fixed dose I-131 therapy for Graves’ disease with an iodine restricted diet. Methods: Consecutive adult patients with GD planned for I-131 therapy were block randomized either to receive instructions regarding dietary iodine restriction or no advice prior to fixed dose (5 mCi) I-131 administration. Urinary iodine excretion rates were assessed at 3 & 6 months to document adequate iodine restiction in diet. Data was analysed using SPSS V 11.0 (Chicago IL USA) software at the end of 6 months regarding development of hypothyroidism, requirement of L-T4 and cure of hyperthyroidism at 3 and 6 months. Results Forty seven patients (13M & 34F) were assessed, 2 were excluded and 45 were randomized (Cases 24 & Controls 21) in the study. 39 patients completed the study and were included in the final analysis. Baseline data was comparable (Table 1). Median Urinary Iodine concentration was 115 and 273 mcg/gm creat (p= 0.001) among cases and controls respectively. Outcomes at the 3rd month were as follows (cases & controls): Euthyroid (10&6:p=0.24), Hypothyroid (3&5:p=0.38) and Hyperthyroid (7&8: p=0.64). Outcomes at six months were as follows (cases & controls): Euthyroid (10&5:p=0.12), Hypothyroid (3&5:p=0.38) and Hyperthyroid (7&9:p=0.43). Of the hypothyroid patients 5 (cases 1 & controls 4: p = 0.13) required thyroxine replacement. Thirteen patients were considered cured among the cases compared to 10 among the controls (p=0.43. Discussion: The increased incidence of autoimmune thyroid disease among genetically susceptible individuals with increasing dietary iodine has been demonstrated both epidemiologically and experimentally. This study did not reveal any statistically significant difference by restricting iodine in on the short term outcome of patients following radioactive iodine therapy for Graves’ disease. A trend for better cure rates and decreased requirement for levothyroxine replacement therapy at 6 months was observed with iodine restriction; this was not statistically significant. This trend, though not significant because of the small sample size maybe related to the increased antigenicity of thyroglobulin with excess iodine moieties. The ongoing autoimmune process would accelerate the development of hypothyroidism in a subset of patients and also result in residual thyrotoxicosis in a second subset. The overall cure rate with 5 mCi of 131I in this study was only a disappointing 51%. The dose used in this study was much lower than conventional fixed dosing regime. A larger sample size with a higher dose of 131-I might have given different results. Conclusions: There was no statistically significant difference in the outcome of patients with dietary iodine restriction following 131I therapy for Graves’ disease but a trend towards better cure rates and decreased requirement for levothyroxine (L-T4) was observed.

Abstract: Objective: To report two siblings with Perrault syndrome and marfanoid body proportions expanding the spectrum of the disease. Case Presentation: The older of the siblings presented initially with hearing loss related to bilateral Sensorineural deafness (SND) (Figure 1). A year later she presented with primary amenorrhea and failure to develop any secondary sexual characteristics. Investigations revealed elevated gonadotropins, a normal karyotype and non visualized ovaries. She was 169cm tall and weighed 56kgs. Her body proportions were Marfanoid with an arm span of 182 cm and an upper segment: lower segment (US: LS) ratio of 0.78:1. She had long slender fingers with a positive wrist and thumb signs. The eye, neurological and cardiovascular systems were normal. The younger sibling had been brought to our clinic at the age of 16 yrs with complaints of primary amenorrhea and lack of secondary sexual development. She also complained of a progressive decline in hearing. She was 162 cm tall and weighed 40kgs with an US: LS ratio of 0.78: 1. She had long slender fingers, high arched palate and positive thumb and wrist signs. She also had elevated gonadotropin levels and normal karyotype and non visualized ovaries. ENT evaluation revealed severe SND. Discussion: The presentation of the siblings with primary amenorrhea, underdeveloped genitalia, normal female karyotype and significant SND was consistent with the diagnosis of Perrault syndrome. The pathogenetic relationship between ovarian dysgenesis and SND is unclear. SND appears to be a part of a more widespread neurological involvement seen in a subset of patients with Perrault syndrome. The other involvement included mental retardation, ataxia, hypotonia, weakness, spastic diplegia and epilepsy and severe peripheral neuropathy. SND may represent the first step of a more widespread neuronal degeneration. Both our patients have no addi- ABSTRACTS – Reproductive Endocrinology – 70 – tional neurological involvement. The findings of abnormal body proportions noted in our siblings have not been previously reported. Both our siblings had abnormal body proportions with high arched palate and long fingers with positive wrist and thumb signs. The genetic basis of PS is still unclear. The disease may represent a heterogeneous group of genetic disorders with multiple gene defects. Preliminary studies on the first PS family reported has mapped the defect to the long arm of chromosome 5. Conclusions: Perrault syndrome represents a spectrum of disorders characterized by 46 XX gonadal dysgenesis and neurological involvement commonly SND. Body proportions and skeletal features of Marfan’s syndrome not reported previously maybe be part of the extended phenotype or may represent a coincidence. As SND may represent the first step to more widespread neurological degeneration patients should to be carefully followed up for development of central and peripheral neurological deficits.

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Abstract: INTRODUCTION: Parathyroid tumours account for a very small percentage of all head and neck neoplasms. The clinical presentation of benign primary hyperparathyroidism differs from that seen in Western series. We attempt to review the presentation and outcomes of patients treated for malignant primary hyperparathyroidism from our centre and highlight the clinical clues to a early pre-operative diagnosis. METHODS: A retrospective review was conducted of all cases of parathyroid carcinoma treated at Christian Medical College (University affiliated teaching hospital) from 1990 – 2005. Five patients were identified. Their demographic characters, clinical presentation, management details, and treatment outcome were obtained from the case records. RESULTS: Five subjects were identified with parathyroid carcinoma. There was a male preponderance and the mean age of presentation was 49 years. The clinical presentation among patients with parathyroid carcinoma and benign primary hyperparathyroidism was similar. Clinically the only significant difference the presence of palpable nodule in 80% of patients with carcinoma compared to 11% among patients with benign primary hyperparathyroidism (p value ). Biochemically all patients with parathyroid carcinoma had hypercalcaemia with elevated serum PTH levels and there was no significant difference compared to benign hyperparathyroidism. Surgery was done in all patients. Intraoperatively evidence of local metastasis to the cervical lymphnodes was seen in 40% off patients. The tumours showed a high predilection to the inferior parathyroid glands.. All subjects underwent radiotherapy with cobalt at mean dose of 50cGy. Recurrence was evident in four subjects .The mean duration of recurrence from the time of primary surgery was about 2 years. One subject had vocal cord involvement and underwent tumour resection from the cords. Bony metastasis was found in one subject as evidenced by hot spots in sestamibi scan. Two of the five patients died and one paient was lost to follow up. CONCLUSION: Parathyroid carcinoma constitutes around 2-3% of patients with primary hyperparathyroidism in our country. The presence of a palpable nodule in the neck is a clinical preoperative clue to the presence of malignancy. Despite routine post operative radiotherapy in our centre the recurrence rate of parathyroid cancer is similar to what is reported in literature.

Abstract: Aim; To characterize the clinical features, biochemistry and imaging features of pancreatic disease in primary hyperparathyroidism ( PHPT) Methods; This study is a retrospective medical record review of thirteen patients who were diagnosed with primary hyperparathyroidism and presented with pancreatic disease among a total of 102 patients with a diagnosis of Primary hyperparathyroidism at Christian Medical College and Hospital Vellore between 1st May 2000 and 30th May 2005. The medical records were reviewed for patient demographics and clinical details. Biochemical records of all the 102 patients with PHPT were retrieved and the radiology of the identified patients were reviewed with a radiologist. Results; Thirteen patients were identified among a cohort of 102 (12.7%) patients with confirmed PHPT. The patients ranged from 22-52 years in age with a median age of 37 years. There were 9 ( 69.2%) males, The common presentation of pancreatic disease was recurrent episodes of upper abdominal pain which was seen in 6 (46.2%) or a single episode of acute pancreatitis was seen in another 6 ( 46.2%) of these patients. One patient (7.7%) among the 13 presented with a painless chronic pancreatitis. Symptomatic Nephrolithiasis was seen in 6 (46.2%) patients along with symptoms of pancreatic disease. Two patients (15.4%) had history of bone pains while 3 (23.1%) had proximal muscle weakness. Two (15.4%) patients had history of pathological fractures and 1 (7.7%) patient had a palpable mass in the neck. Ten patients (76.9%) had enlarged pancreas and the rest had normal sized pancreas. Intraparenchymal calcification was seen in 5 (38.5%) and intraductal calculi seen in 4 (30.8%) of the 12 patients. The main pancreatic duct could not be visualized in 2 (15.4%) of the 12 patients. Conclusions; The present series reveals an association of over 12% that is larger than previous series. In the present series PHPT presents as pancreatic disease in much younger patients with a male preponderance. The commonest manifestation of pancreatic disease with PHPT is the history of recurrent upper abdominal pain (50%). The mean calcium values were significantly higher among patients with PHPT and pancreatic disease compared to patients with PHPT without pancreatic involvement. A Bulky pancreas and a dilated main pancreatic duct were the commonest finding on imaging.

Abstract: Context : Primary Hyperparathyroidism (PHPT) in India and in other developing countries is still recognized by its classic manifestations1,2,3. A significant proportion of patients with PHPT with symptomatic disease have long term sequelae of the disease some of which require lifelong therapy in addition to the removal of the tumour. Objectives : We describe data from 111 patients with PHPT seen over a period of five years. We attempt to characterize the presentation of PHPT and describe the long term implications of PHPT among operated patients. Design and setting : This study is a retrospective medical record review of all patients who were diagnosed with PHPT at tertiary care medical centre over 5 years. The biochemical diagnosis of PHPT was made when persistent elevated calcium levels with an increased level of circulating intact Parathyroid Hormone (iPTH). The statistical analysis was carried out using SPSS version 11.0 Results A total of 111 patients were studied. There were 57 women and 54 men. The median age of presentation was 42 years. Only six (5%) patients were asymptomatic. Of the symptomatic patients, the common presentations were bone pains in 44 (40%), nephrolithiasis in 33(30%) and pancreatitis in 15(13%). The mean lag time from the initial reported symptom to the time of diagnosis of PHPT was 33 42 months. Long term implications of treated disease Eighteen (16%) patients had evidence of renal parenchymal calcification. Twenty three (21%) patients had decreased glomerular filtration rate with increased creatinine values because of either bilateral hydronephrosis and/or nephrocalcinosis. An average of 2.3 urological procedures was done on 33 patients with presenting complaints of nephrolithiasis. Nine patients (8%) had evidence of chronic pancreatitis when the diagnosis of primary hyperparathyroidism was made. Three of these patients had secondary diabetes and two had evidence of pancreatic malabsorption. Conclusions Primary Hyperparathyroidism is an uncommon disease in India with over 95% of patients presenting with significant symptoms. The mean age of presentation is at least two decades earlier than what is observed in Western series. The mean lag time to diagnosis was around 3 years. Over one fifth of patients had long-term complications of PHPT either in the form of renal parenchymal calcification or chronic pancreatitis.

Abstract: Aim; To characterize the clinical features, biochemistry and imaging features of pancreatic disease in primary hyperparathyroidism ( PHPT) Methods; This study is a retrospective medical record review of thirteen patients who were diagnosed with primary hyperparathyroidism and presented with pancreatic disease among a total of 102 patients with a diagnosis of Primary hyperparathyroidism at Christian Medical College and Hospital Vellore between 1st May 2000 and 30th May 2005. The medical records were reviewed for patient demographics and clinical details. Biochemical records of all the 102 patients with PHPT were retrieved and the radiology of the identified patients were reviewed with a radiologist. Results; Thirteen patients were identified among a cohort of 102 (12.7%) patients with confirmed PHPT. The patients ranged from 22-52 years in age with a median age of 37 years. There were 9 ( 69.2%) males, The common presentation of pancreatic disease was recurrent episodes of upper abdominal pain which was seen in 6 (46.2%) or a single episode of acute pancreatitis was seen in another 6 ( 46.2%) of these patients. One patient (7.7%) among the 13 presented with a painless chronic pancreatitis. Symptomatic Nephrolithiasis was seen in 6 (46.2%) patients along with symptoms of pancreatic disease. Two patients (15.4%) had history of bone pains while 3 (23.1%) had proximal muscle weakness. Two (15.4%) patients had history of pathological fractures and 1 (7.7%) patient had a palpable mass in the neck. Ten patients (76.9%) had enlarged pancreas and the rest had normal sized pancreas. Intraparenchymal calcification was seen in 5 (38.5%) and intraductal calculi seen in 4 (30.8%) of the 12 patients. The main pancreatic duct could not be visualized in 2 (15.4%) of the 12 patients. Conclusions; The present series reveals an association of over 12% that is larger than previous series. In the present series PHPT presents as pancreatic disease in much younger patients with a male preponderance. The commonest manifestation of pancreatic disease with PHPT is the history of recurrent upper abdominal pain (50%). The mean calcium values were significantly higher among patients with PHPT and pancreatic disease compared to patients with PHPT without pancreatic involvement. A Bulky pancreas and a dilated main pancreatic duct were the commonest finding on imaging.

Abstract: Objectives : To calculate the sensitivity, specificity and positive predictive value of 99 Tc Sestambi scan in the localization of parathyroid disease and to calculate the cost benefit of routine pre-operative scan. Methods : This is a retrospective medical review of 161 patients who underwent the 99 Tc Sestambi scan at Christian Medical College and Hospital Vellore, India between 1st May 2000 to 30th May 2005. The sensitivity, specificity and positivity predictive value was calculated using standard formulae. True positives were defined as patients with confirmed primary hyperparathyroidism (PHPT) on pathological examination. True negatives were patients with negative biochemical evaluation and sestamibi scan which was negative. False positive were patients with positive scans and no biochemical features of HPT. False negatives were patients with a negative scan and biochemical hyperparathyroidism with a histological diagnosis of PHPT. Results : The sensitivity of 99mTc-Sestamibi for accurate unambiguous localization of parathyroid adenoma is 76.19% with a specificity of 78.19%. The positive predicative value for 99mTc-Sestamibi for accurate and unambiguous localization is 84.2%. There is better sensitivity for lesions which are inferior and on the left side of the neck. The cost benefit analysis revealed benefits of preoperative scanning of all patients undergoing bilateral neck exploration. Conclusion : 99mTc-Sestamibi remains a valuable tool for localization of preoperative parathyroid disease. In the present clinical setting it may not be sensitive enough to replace bilateral neck exploration with more limited procedures. However the cost benefit analysis confirms the benefits of routine pre-op scanning for identifying ectopically based adenomas.

Abstract: Objective Hypokalemic periodic paralysis (HPP) represents a heterogeneous group of disorders that presents with acute muscular weakness. Familial periodic paralysis is the commonest cause of hypokalemic periodic paralysis in Caucasians and thyrotoxic paralysis in the Orientals. In this analysis, we have complied the etiological factors that appear to be more dominant in Indian population. Methods The clinical and laboratory data of patients who presented with periodic paralysis over a 6-year period is analysed. Patients were investigated for possible secondary causes of hypokalemia. Results Of the 31 patients with HPP, 19 were males 12 were females. They had a mean age of 34.5 years (11-68 years). Thirteen of the 31 patients who presented with hyperparathyroidism (HPP) had renal tubular acidosis (RTA) (42%), 12 had primary hyperaldosteronism (42%). There were two subjects with thyrotoxic periodic paralysis, two with sporadic periodic paralysis and one with Gitelman’s syndrome. Of the thirteen patients with renal tubular acidosis, ten had proximal RTA and three had distal RTA. Three patients with RTA had Sjogren’s syndrome. The patients diagnosed to have RTA had significantly lower serum bicarbonate (18.7 ± 4.6 vs. 29.6 ± 5.0, p < 0.05) and higher levels of chloride (107.5 ±6.0 vs. 99.5 ± 3.4, p < 0.05) compared to those with a diagnosis of primary hyperaldosteronism although the potassium values were similar (2.4 ± 0.65 vs. 2.26 ± 0.48, p=0.43). All patients with primary hyperaldosteronism and hypertension at presentation and were proven to have adrenal adenomas. Two patients with thyrotoxic periodic paralysis underwent radioactive iodine ablation of thyroid. Conclusions A significant number of patients in the study had secondary and potentially reversible causes of HPP. A detailed work up for secondary causes should be undertaken in Indian patients with HPP. Identifying the cause of HPP can lead to optimized management protocols.

Abstract: Background: Insulinoma is a disorder affecting one in 100,000 of the general population. The clinical profile of insulinomas among Indian patients has not been characterized. Aim of the study: To study the clinical characteristics, biochemical and radiological parameters and treatment aspects of adult patients with insulinomas. Methodology: A retrospective analysis of 18 patients with insulinoma who were treated from 1992 to 2004 in our institution was performed. The data was analyzed using a 9.0 SPSS software package. Results: The mean age of the patients was 43.11 years (standard deviation 15.24) with a male predominance (range 20-70 years). The commonest presenting feature was recurrent loss of consciousness (72%). The mean plasma glucose on a 72 hour fast was 35 mg /dl (standard deviation 5.82). The mean insulin to glucose ratio was 1.67 (standard deviation 1.08). Two patients had features of Multiple Endocrine Neoplasia type I syndrome (11 %).Fourteen patients underwent surgery and 4 patients opted for medical treatment. All tumours that were identifiable on histopathology were palpable during the intraoperative period. Histopathology revealed a definite tumour in 10 out of 14 patients and in four patients, there was no evidence of abnormal pathology. Out of those with tumours, only one was found to have malignant characteristics on histopathology. The most common location of the tumour was in the head and uncinate process of the pancreas (43%). Accurate preoperative tumour localization was possible in 11 patients (79%). Computerized Tomography had a sensitivity of 62.5% and positive predictive value of 100% in localizing tumours. In 12 subjects out of 14 who underwent surgery the blood glucose levels normalized. Two out of four patients who opted for medical treatment are currently asymptomatic and the remaining 2 patients have been lost to follow up. Conclusions: In contrast to other series, the presentation of Insulinoma revealed a male preponderance. In patients who underwent surgery, preoperative localization by radiological techniques was more successful than expected. CT scan had a good positive predictive value in localizing tumours. Of the patients who underwent surgery, a large majority are in remission.