Fetal Hyperthyroidism: Intrauterine Treatment with Carbimazole in Two Siblings.

Hyperthyroidism can manifest very early in fetal life (fetal thyrotoxicosis) or immediately after birth (neonatal thyrotoxicosis). The authors describe outcome of pregnancies in a woman with Graves' disease who received medical management and underwent subtotal thyroidectomy. The first pregnancy resulted in macerated stillbirth at 32 wk. Fetal tachycardia was followed by intrauterine death at 30 wk in the second pregnancy and macerated stillbirth at 26 wk in the third pregnancy. Fetal tachycardia was detected at 17 wk in the fourth pregnancy. Treatment with carbimazole along with thyroxine was followed by a live birth at 35 wk; but the baby developed severe fatal neonatal thyrotoxicosis with crisis on day 9 and died on day 12. Fetal tachycardia was noted in the fifth pregnancy as well and she was treated with carbimazole and thyroxine. She delivered a male baby at 37 wk. He developed neonatal hypothyroidism on day 8 which was controlled with thyroxine.

Precocious puberty--perspectives on diagnosis and management.

The term 'precocious puberty' signifies the onset of secondary sexual characters before the age of 9 y in boys and 8 y in girls. Menarche before 9.5 y is also considered precocious. These definitions are constantly evolving due to the secular trends observed all over the world. It is crucial to decide whether the child has central (gonadotropin-dependent, GDPP) or peripheral (gonadotropin-independent, GIPP) form of precocious puberty. Some benign conditions such as premature thelarche and premature pubarche may mimic precocious puberty. A systematic approach with detailed history and clinical examination helps to arrive at a diagnosis in most cases. An underlying neurologic disorder is more likely in a very young boy. Basal LH level is the best screening test to diagnose GDPP. LH level less than 0.1 IU/L by a very sensitive assay indicates prepubertal stage. Stimulation tests using gonadotropin releasing hormone (GnRH) or its analog (GnRHa), leuprolide help to confirm the diagnosis of GDPP. High resolution MRI of brain helps to detect abnormalities in hypothalamus and pituitary region. GnRH analogs (GnRHa) are the only effective treatment for GDPP at present. In girls, breast size may regress; menses ceases and vaginal mucosa becomes non-estrogenized. In boys testicular volumes remain static or decrease and genital growth regresses. The effects of GnRH analogs are reversible on discontinuation of therapy, with restoration of normal function within 3 mo after stopping treatment. Treatment of GIPP however is far from satisfactory.

Contemporary issues in precocious puberty.

Precocious puberty poses significant diagnostic and therapeutic challenge to the physician. Recent advances in the understanding of pathophysiology of precocious puberty have resulted in improved management. Timely intervention is mandatory to achieve successful outcome. The identification of critical role of KISS-1-kisspeptin-GPR54 system has gone a long way to provide an insight into pubertal physiology. It is likely that the system would become an important diagnostic and therapeutic target in children with precocious puberty. Epidemiological studies point toward earlier thelarche. This is, however, associated with slower progression as the age of menarche is static. These changes have led to suggestions of lowering the age cutoffs for precocious puberty in girls. New developments in assessment of precocious puberty including gonadotropin releasing hormone (GnRH) agonist test have made characterization of precocious puberty easier. Longstanding GnRH analogs have become the mainstay of treatment of gonadotropin-dependent precocious puberty, while aromatase inhibitors and inhibitors of sex hormone action are increasingly being used in gonadotropin-independent precocious puberty.

An objective assessment of proximal and distal facial nerve exploration during superficial parotidectomy.

AIM: This study was undertaken to compare the proximal and distal facial nerve exploration approach during superficial parotidectomy. MATERIALS AND METHODS: A retrospective analysis of patients who underwent superficial parotidectomy at our center was conducted. Cases were divided into those who underwent superficial parotidectomy using distal facial nerve exploration and those who underwent standard proximal facial nerve exploration. Statistical comparisons of intraoperative blood loss and margin status (negative, focally, positive) were conducted between these two approaches. RESULTS: A total of 39 patients underwent superficial parotidectomy at our center between 2008 and 2010. The technique used in most of the cases was conventional proximal nerve exploration technique (29 cases). Distal exploration of the buccal branch was undertaken only in 10 cases, on account of difficulty in locating the main trunk intraoperatively due to the presence of postinflammatory fibrosis. The average patient age was 48 years with a female preponderance (67%). Both the techniques consumed almost same average operative time (2.4 hours) and average intraoperative blood loss (68.0 cc vs 25.4 cc) was more in the cases where proximal nerve exploration was resorted (S.E (d) = 0.89). No significant difference in surgical margin status was noticed between the two techniques (P > 0.05). CONCLUSION: Both the techniques are efficient without compromising the surgical margins, but the average intraoperative blood loss is less in distal facial nerve exploration technique.

Clinical profile of primary hyperparathyroidism from western India: a single center experience.

BACKGROUND: Primary hyperparathyroidism (PHPT) has a variable clinical presentation and symptomatic PHPT is still the predominant form of the disease in India. Data from western India is lacking. AIM: To present the clinical profile of PHPT from western India. SETTINGS AND DESIGN: This retrospective study was conducted at a tertiary care referral center. MATERIALS AND METHODS: We analyzed the clinical presentation, biochemical, radiological features, and operative findings in adult patients with PHPT (1986-2008) and compared with our published data of children and adolescent patients with PHPT. STATISTICAL ANALYSIS: was done with SPSS 16 software. RESULTS: Seventy-nine patients (F: M-2:1) with age ranging from 21 to 55 years (mean 33.5+/-8.82) were analyzed. Skeletal manifestations (75.5%), renal calculi (40.5%) and proximal muscle weakness (45.5%) were the most common symptoms of presentation with mean duration of symptoms being 33.70 (median: 24, range 1-120) months. Biochemical features included hypercalcemia (total corrected calcium 12.55+/-1.77 mg/dl), low inorganic phosphorus (1.81+/-0.682 mg/dl), elevated total alkaline phosphatase (mean: 762.2; median: 559; range: 50-4930IU/L) and high parathyroid hormone (PTH) (mean+/-SD: 866.61+/-799.15; median: 639.5; range: 52-3820 pg/ml). Preoperative localization was achieved in 74 patients and single adenoma was found during surgery in 72 patients. Hungry bone disease was seen in 30.3% and transient hypoparathyroidism developed in 62% patients. In comparison to PHPT in children there were no significant differences with regard to clinical, laboratory and radiological features. CONCLUSIONS: PHPT in western India is symptomatic disorder with skeletal and renal manifestations at a much younger age. Clinical profile of PHPT in children is similar to that of adults.

Study of bone mineral density in resident doctors working at a teaching hospital.

CONTEXT: The erratic lifestyle of resident doctors may affect their serum 25-hydroxy vitamin D [25-(OH)D] levels and bone mineral density (BMD). AIM: To study BMD and the effect of environmental factors on it in resident doctors. SETTINGS AND DESIGN: Prospective, cross-sectional study conducted in a tertiary healthcare centre. MATERIALS AND METHODS: BMD was obtained by dual-energy X-ray absorptiometry and was correlated with various factors including weight, height, body mass index (BMI), sun exposure, physical activity, parathyroid hormone, 25-(OH)D, dietary factors. Statistical Analysis : SPSS software Version 10 (Unpaired t test was used to compare BMD of different groups and Pearson's correlation coefficient was used to calculate correlation). RESULTS: Two hundred and fourteen apparently healthy resident doctors were enrolled in the study. Based on Caucasian normative data, osteopenia was noted in 104 (59.7%) males and 27 (67.5%) females. Thirty-two (18.39%) males and five (12.5%) females had osteoporosis. The BMD values of males were 0.947+/-0.086, 0.911+/-0.129 and 1.016+/-0.133 at lumbar spine, femur neck and total hip while those in females were 0.981+/-0.092, 0.850+/-0.101 and 0.957+/-0.103 respectively. BMD of our cohort was lesser by 12.5-18.2% and 4.2-14.5% than the Caucasian and available Indian figures, respectively. BMD had significant positive correlation with weight, height, BMI, physical activity, and dietary calcium phosphorus ratio. 25-(OH)D levels were insufficient in 175 (87.5%) subjects but had no correlation with BMD. CONCLUSIONS: Young healthy resident doctors had significantly lower BMD, contributors being lower BMI, lower height, reduced bioavailability of dietary calcium and inadequate physical activity. Deficiency of vitamin D did not contribute to low BMD.

Physical growth in children with reflux nephropathy with normal or mildly impaired renal function.

Ten children aged 11 months to 10 years (means 5.7 years) with reflux nephropathy, vesicoureteric reflux (VUR) and normal or mildly impaired renal function having GFR more than 50 ml/min/1.72 m(2), were included in the study. The hematological and biochemical parameters were within normal limits. Height standard deviation score (HZ score) was reduced at entry and, decreased further during follow-up (-2.2 and -2.6 at 0 and 12 months, respectively). Weight for height index (WHI) improved significantly (p=0.0004) during follow-up. The basal and stimulated peak growth hormone levels of these patients were found to be elevated, 18.53 +/- 11.36 microg/L and 34.20 +/- 5.86 microg/L, respectively. The IGF-1 levels were low ranging from 45.00 to 84.40 ng/dl (mean +/- SD 61.54 +/- 10.21 ng/dl) compared to 51.80 to 247.50 ng/dl (mean +/- SD111.20 +/- 70.24 ng/dl) in age and sex matched controls, indicating partial insensitivity to growth hormone.

The value of an acute octreotide suppression test in predicting response to long-term somatostatin analogue therapy in patients with acromegaly.

CONTEXT: The usefulness of the acute octreotide test in the selection of patients with acromegaly for chronic somatostatin depot analogues treatment is controversial. AIMS: To determine the efficacy of acute octreotide suppression test (OST) in predicting response to long-term somatostatin analogue (Octreotide-long-acting repeatable, OCT-LAR) therapy in patients with acromegaly. SETTINGS AND DESIGN: Prospective study (2006-2007) conducted at a tertiary healthcare centre in western India. MATERIALS AND METHODS: Sixteen drug-naive patients with active acromegaly (postoperative+/-post radiotherapy) underwent 50 microg subcutaneous OST. Ten patients were treated with OCT-LAR for one year. Remission was defined as a nadir growth hormone (GH) < 1 ng/ml during 75 g oral glucose tolerance test (OGTT) (0, 10, 30, 60, 120, 180 min) and normal age, sex-matched insulin-like growth factor 1 (IGF1) levels. STATISTICAL ANALYSIS: SPSS Software Version 11 was used for data analysis. RESULTS: Using GH cutoff. CONCLUSIONS: Nadir GH < 1 ng/ml following an OST is a useful predictive marker of achieving disease remission with long-term OCT-LAR therapy.

Fractures in the Maxillofacial Region: A Four Year Retrospective Study.

INTRODUCTION: The incidence of maxillofacial injuries is on the rise due to motor vehicle accidents and increased incidence of violence in recent times. The aim of this retrospective study was to determine the incidence, aetiology, the pattern of fractures, their management with open reduction and internal fixation (ORIF) and complications, if any. METHODS: A retrospective analysis of 621 fractures in 361 patients managed by ORIF over a four year period was carried out. RESULT: The average age of patients was 24.3 years with a male to female ratio of 21.2:1. Panfacial fractures comprised 4.7%, frontal bone fractures 8.9%, orbital fractures 0.7%, naso-orbito-ethmoid complex (NOE) fractures 0.7%, zygomatic complex fractures 23.5%, fracture maxilla 11.5% and mandibular fractures 52.2% of all facial fractures. All the cases were successfully managed by ORIF under general anaesthesia (GA). Complications were noticed in 6.8% of cases in the form of reactive implants in 3.6%, deranged occlusion in 1% and infection at operated site in 1% cases which were managed satisfactorily. CONCLUSION: The findings of this study reveal sharp annual increase in the number of cases of maxillofacial trauma. Road traffic accidents (RTA) were the commonest cause and the age group most affected was between 20-25 years. ORIF of these fractures was chosen for its obvious advantages of direct anatomical reduction, early return to function and minimal complications.

Management protocol for anaphylaxis.

There is no universal agreement on the definition of anaphylaxis or the criteria for establishing its diagnosis, although it has been known to the field of emergency medicine for more than 100 years. Two meetings were convened by the National Institute of Allergy and Infectious Disease and Food Allergy and Anaphylaxis Network in April 2004 and July 2005. Representatives from 16 different organizations and government bodies, including representatives from developed nations, continue working toward a universally accepted definition, criteria for diagnosis, and management of anaphylaxis. This article presents the latest concepts on anaphylaxis in the literature including the research needs in this area.

Autoimmune hypophysitis: a single centre experience.

INTRODUCTION: Autoimmune hypophysitis (AH) is a rare primary autoimmune inflammatory disorder involving the pituitary gland. METHODS: A retrospective analysis of the clinical features and outcome of patients diagnosed with AH between 1988 and 2006, was carried out. RESULTS: 15 patients (14 females and one male) with AH were identified. Three patients presented in the peripartum period. Headache, vomiting and visual field defects, suggestive of an expanding sellar mass, were the most common presenting symptoms (67 percent). The most common deficient hormone was adrenocorticotropic hormone (ACTH) (67 percent), followed by thyroid stimulating hormone (53 percent) and gonadotropins (40 percent). Imaging revealed a definite, enhancing sellar mass in 87 percent of the patients and stalk thickening in 33 percent of the patients. Three patients underwent surgery. On serial monitoring, the sellar mass regressed or disappeared spontaneously without any immunosuppressive treatment in the other ten patients with a definite sellar mass. CONCLUSION: We report a higher female to male ratio and a lower incidence of peripartum cases in our series. Symptoms of mass effect were the most common presentation, while ACTH was the most commonly-deficient hormone. Surgery was rarely needed, and most patients experienced a spontaneous resolution of the mass.

Prevalence of nonalcoholic fatty liver disease in patients with type 2 diabetes mellitus.

OBJECTIVES: Nonalcoholic fatty liver disease (NAFLD) is commonly associated with type 2 diabetes mellitus (DM) though its prevalence is not well studied. We conducted a prospective study of prevalence and risk factors of NAFLD in patients with type 2 diabetes. RESEARCH DESIGN AND METHODS: 204 type 2 DM patients attending an out-patient diabetic clinic underwent abdominal sonography. Ninty of 127 patients with fatty infiltration on ultrasound consented for liver biopsy, clinical and biochemical workup. RESULTS: Eighty seven percent had NAFLD on histology with 62.6% steatohepatitis and 37.3% fibrosis. Age, duration of diabetes mellitus, degree of glycemic control, body mass index, waist circumference, family history of diabetes mellitus, did not predict the presence or severity of NAFLD or fibrosis. Serum alanine aminostranferase (ALT) and alkaline phosphatase levels, though within normal limits, were significantly higher in patients with steatohepatitis. Prevalence of NASH increased with increase in the components of the metabolic syndrome. Serum AST/ALT ratio were also significantly higher (p-0.049) in patients with severe fibrosis. All patients with severe fibrosis had metabolic syndrome. CONCLUSIONS: Prevalence of NAFLD and NASH in our cohort of type 2 DM patients is high and increases with multiple components of metabolic syndrome. NASH and advanced fibrosis can occur in diabetic patients without any symptoms, signs or routine laboratory test abnormalities.