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1.  Paradoxical response to dexamethasone and spontaneous hypocortisolism in Cushing's disease 
BMJ Case Reports  2013;2013:bcr2012008035.
Paradoxical response to dexamethasone and spontaneous development of hypocortisolism are rare features of Cushing's disease. We report a 13-year-old boy with Cushing's disease owing to a pituitary macroadenoma. On initial evaluation, he had partial suppression of serum cortisol by dexamethasone. He developed transient hypocortisolism after first adenomectomy, but the disease recurred after 1 year. Repeat evaluation showed recurrent hypercortisolism and paradoxical response to dexamethasone. He underwent second surgery and, postoperatively, hypercostisolism persisted even after 2 years of surgery. Repeat evaluations after 8 years of second surgery revealed persistent hypocortisolism despite residual tumour of same size and similar plasma adrenocorticotropic hormone (ACTH) levels. We have also shown that the paradoxical increase in serum cortisol was preceded by a paradoxical increase in ACTH. The paradoxical response persisted despite hypocortisolism. This patient with Cushing's disease had two very rare features: paradoxical response to dexamethasone and spontaneous development of hypocortisolism.
doi:10.1136/bcr-2012-008035
PMCID: PMC3604017  PMID: 23365169
2.  Morbidity pattern in hospitalized under five children with sickle cell disease 
Background & objectives:
Children with sickle cell disease require more frequent hospital care and younger children (<5 yr of age) are more vulnerable to mortality. There are limited data on the events leading to hospitalizations and death in younger children with sickle cell disease from India. This study was, therefore, undertaken to evaluate the morbidity pattern in hospitalized under five children with sickle cell disease in a tertiary care hospital in Maharashtra, India.
Methods:
This was a prospective observational study carried out from July 2007 to June 2009. Hospitalized children below five years of age with sickle cell disease were enrolled for the study and evaluated for morbid event/s leading to hospitalization. Haematological indices were noted at baseline (most recent past when patient was not acutely sick) and at the time of hospitalization.
Results:
Eighty five children with sickle cell disease were hospitalized during the study period. Hospitalization with acute febrile illness (31%) was the most common morbid event followed by severe anaemia (30%) and acute painful events (20%). Majority (62%) of the events occurred between August and October. Forty five patients had foetal haemoglobin (HbF) more than 20 per cent (26.80±4.81%) and morbidity was significantly less in these patients.
Interpretation & conclusion:
Acute febrile illness was the most common morbid event followed by severe anaemia and acute painful event hospitalized children with sickle cell disease. There was significant seasonal variation with maximum events occurring in the monsoon season.
PMCID: PMC3818593  PMID: 24135175
Bacteraemia; children; haemoglobin; infection; morbidity; sickle cell disease
3.  Aortoarteritis: Could it be a form of catecholamine-induced vasculitis? 
Catecholamine-induced vasculitis is a well known but rarely described entity. However, aortoarteritis as a manifestation of catecholamine-induced vasculitis is not described in the literature. We have reported two patients in whom pheochromocytoma coexisted with aortoarteritis. Both patients were young females with history of bilateral pheochromocytomas in more than one first-degree relative. Both patients also had bilateral adrenal pheochromocytomas (second patient also had paraganglioma at left renal hilum) with elevation of plasma free normetanephrine levels. We conclude that there may be an association between pheochromocytoma and aortoarteritis, and that catecholamine excess may have a role in the etiopathogenesis of aortoarteritis in these patients.
doi:10.4103/2230-8210.107874
PMCID: PMC3659888  PMID: 23776874
Aortoarteritis; catecholamine excess; pheochromocytoma
4.  Coexistence of pheochromocytoma/praganglioma and renal artery stenosis 
Renal artery stenosis (RAS) often coexists with pheochromocytoma (Pheo)/paraganglioma (PGL) and often alters the management of patients with Pheo/PGL. We have studied the prevalence of RAS in our Pheo/PGL patients. The study included 70 consecutive, histopathologically proven Pheo/PGL patients from a tertiary health care center. In 60 patients, tumors were limited to adrenal glands (54 unilateral and 6 bilateral) while other 10 patients had extra-adrenal abdominal tumors. Five patients had RAS with an overall prevalence of 14%. Only two out of 60 patients with Pheo had RAS with a low prevalence of 3.3% while three out of 10 patients with extra-adrenal abdominal PGL had RAS with a prevalence of 30%. To conclude, RAS commonly coexists with Pheo/PGL, more often with extra-adrenal PGL.
doi:10.4103/2230-8210.103022
PMCID: PMC3510927  PMID: 23226653
Paraganglioma; pheochromocytoma; renal artery stenosis
5.  Hypophosphatemic rickets 
Hypophosphatemic rickets is a disorder of bone mineralization caused due to defects (inherited/acquired) in the renal handling of phosphorus. This group includes varied conditions, X-linked hypophosphatemic rickets being the most common inheritable form of rickets. The other common forms are autosomal dominant hypophosphatemic rickets and tumor-induced osteomalacia. Although these conditions exhibit different etiologies, increased phosphatonins form a common link among them. Fibroblast growth factor 23 (FGF23) is the most widely studied phosphatonin. Genetic studies tend to show that the phosphorus homeostasis depends on a complex osteo-renal axis, whose mechanisms have been poorly understood so far. Newer disorders are being added as the mechanisms in this axis get discovered. This review focuses on the clinical, biochemical, genetic features and management of hypophosphatemic disorders leading to defective mineralization.
doi:10.4103/2230-8210.93733
PMCID: PMC3313733  PMID: 22470852
1; 25(OH)2D3; autosomal dominant hypophosphatemic rickets; fibroblast growth factor 23; hypophosphatemia; tumor-induced osteomalacia; X-linked hypophosphatemic rickets
6.  Renal manifestations of primary hyperparathyroidism 
Primary hyperparathyroidism (PHPT) is associated with nephrolithiasis and nephrocalcinosis. Hypercalciuria is one of the multiple factors that is implicated in the complex pathophysiology of stone formation. The presence of a renal stone (symptomatic or asymptomatic) categorizes PHPT as symptomatic and is an indication for parathyroid adenomectomy. Progression of nephrocalcinosis is largely reversible after successful surgery, but the residual risk persists. PHPT is also associated with declining renal function. In case of asymptomatic mild PHPT, annual renal functional assessment is advised. Guidelines suggest that an estimated glomerular filtration rate (eGFR) < 60 ml / minute / 1.73 m2 is an indication for parathyroid adenomectomy. This article discusses how to monitor and manage renal stones and other related renal parameters in case of PHPT.
doi:10.4103/2230-8210.93745
PMCID: PMC3313745  PMID: 22470864
Nephrolithiasis; primary hyperparathyroidism; renal cyst; renal stones
7.  Cushing's syndrome: Stepwise approach to diagnosis 
The projected prevalence of Cushing's syndrome (CS) inclusive of subclinical cases in the adult population ranges from 0.2–2% and it may no longer be considered as an orphan disease (2–3 cases/million/year). The recognition of CS by physicians is important for early diagnosis and treatment. Late-night salivary cortisol, dexamethasone suppressiontesti, or 24-h urine free cortisol are good screening tests. Positively screened cases need stepwise evaluation by an endocrinologist. This paper discusses the importance of screening for CS and suggests a stepwise diagnostic approach to a case of suspected hypercortisolism.
doi:10.4103/2230-8210.86974
PMCID: PMC3230095  PMID: 22145134
Cushing's syndrome; dexamethasone suppression tests; hypercortisolism; salivary cortisol; urinary free cortisol
8.  Prevalence and Impact of Thyroid Disorders on Maternal Outcome in Asian-Indian Pregnant Women 
Journal of Thyroid Research  2011;2011:429097.
Aims. To establish the prevalence and the effect of thyroid dysfunction on pregnancy outcomes in Asian-Indian population. Subjects and Methods. The study cohort comprised of 483 consecutive pregnant women in the first trimester attending the antenatal clinic of a tertiary center in Mumbai, India. Thyroid hormone levels and thyroid peroxidase antibody were estimated. Patients with thyroid dysfunction were assessed periodically or treated depending on the severity. Subjects were followed until delivery. Results. The prevalence of hypothyroidism, Graves' disease, gestational transient thyrotoxicosis, and thyroid autoimmunity (TAI) was 4.8% (n = 24), 0.6% (n = 3), 6.4 % (n = 31), and 12.4% (n = 60), respectively. Forty percent of the hypothyroid patients did not have any high-risk characteristics. Hypothyroidism and TAI were associated with miscarriage (P = 0.02 and P = 0.001, resp.). Conclusions. The prevalence of hypothyroidism (4.8%) and TAI (12.4%) is high. TAI and hypothyroidism were significantly associated with miscarriage.
doi:10.4061/2011/429097
PMCID: PMC3140706  PMID: 21789274
9.  Prevalence of upper airway obstruction in patients with apparently asymptomatic euthyroid multi nodular goitre 
Aims:
To study the prevalence of upper airway obstruction (UAO) in “apparently asymptomatic” patients with euthyroid multinodular goitre (MNG) and find correlation between clinical features, UAO on pulmonary function test (PFT) and tracheal narrowing on computerised tomography (CT).
Materials and Methods:
Consecutive patients with apparently asymptomatic euthyroid MNG attending thyroid clinic in a tertiary centre underwent clinical examination to elicit features of UAO, PFT, and CT of neck and chest.
Statistical Analysis Used:
Statistical analysis was done with SPSS version 11.5 using paired t-test, Chi square test, and Fisher's exact test. P value of <0.05 was considered to be significant.
Results:
Fifty-six patients (52 females and four males) were studied. The prevalence of UAO (PFT) and significant tracheal narrowing (CT) was 14.3%. and 9.3%, respectively. Clinical features failed to predict UAO or significant tracheal narrowing. Tracheal narrowing (CT) did not correlate with UAO (PFT). Volume of goitre significantly correlated with degree of tracheal narrowing.
Conclusions:
Clinical features do not predict UAO on PFT or tracheal narrowing on CT in apparently asymptomatic patients with euthyroid MNG.
doi:10.4103/2230-8210.83351
PMCID: PMC3169865  PMID: 21966649
Multi nodular goitre; pulmonary function test; upper airway obstruction

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