Deep Dutta

Percutaneous ethanol ablation in managing predominantly cystic thyroid nodules: An eastern India perspective

Aims: Percutaneous aspiration and ethanol injection (PEI) is effective in managing predominantly cystic (>50% cystic) thyroid nodules with efficacy ranging from 38-85%. We aimed to evaluate efficacy, safety, and factors determining outcomes of PEI in managing simple cystic (purely cystic) vs. complex cystic (having both cystic and solid components) thyroid nodules. Materials and Methods: Predominantly cystic thyroid nodules, without any ultrasonography and/or fine needle aspiration, evidence of malignancy were aspirated ultrasonography guided. Sterile 100% ethanol (50-100% volume aspirated) was injected and reviewed monthly for 3 months and thereafter 3 monthly. Responders were defined as ≥ 50% reduction in nodule volume. Results: Sixty-five patients out of 152 considered underwent PEI. Sixty patients [simple cystic (42) and complex cystic (18)] with mean follow-up of 12.3 ± 2.88 months were analyzed. Response rate of PEI was 78.33% [simple cystic (92.86%) and complex cystic (44.44%) nodules; P < 0.001]. Also, 31.67% patients achieved remission at 1st month. And, 46.67% patients achieved remission between 1-6-months follow-up. Kaplan Meier analysis showed significantly improved outcomes in patients with simple cystic nodules (P < 0.001). Cox-regression revealed type of nodule (simple cystic vs. complex cystic) to be predictive of outcome (P = 0.034). Complex cystic nodules were 67.6% less likely to go into remission, compared to simple cystic nodules. Baseline nodule size, aspirate, or volume of ethanol injected did not predict outcome. Conclusions: PEI is safe and should be treatment of choice for simple cystic thyroid nodules. PEI for complex cystic thyroid nodules are associated with lower response, increased recurrence, and need for repeated PEI.

Primitive neuroectodermal tumor of adrenal: clinical presentation and outcomes.

Primitive neuroectodermal tumor (PNET) of adrenal is an extremely rare tumor of neural crest origin. A nonfunctional left adrenal mass (14.6 × 10.5 × 10.0 cm) on computed tomography (CT) was detected in a 40-year-old lady with abdominal pain, swelling, and left pleural effusion. She underwent left adrenalectomy and left nephrectomy with retroperitoneal resection. Histopathology revealed sheets and nest of oval tumor cells with hyperchromatic nuclei, prominent nucleoli, scanty cytoplasm, brisk mitotic activity, necrosis, lymphovascular invasion, capsular invasion, and extension to the surrounding muscles; staining positive for Mic-2 (CD-99 antigen), vimentin, synaptophysin, and Melan-A. Thoracocentesis, pleural fluid study, and pleural biopsy did not show metastasis. She responded well to vincristine, adriamycin, and cyclophosphamide followed by ifosfamide and etoposide (IE). This is the first report of adrenal peripheral PNET (pPNET) from India. This report intends to highlight that pPNET should be suspected in a patient presenting with huge nonfunctional adrenal mass which may be confused with adrenocortical carcinoma.

Oxidative Stress-Associated Neuroretinal Dysfunction and Nitrosative Stress in Diabetic Retinopathy

OBJECTIVEnThe present study was intended to investigate whether oxidative stress is the key regulator to alter neuroretinal biochemical homeostasis and in turn aggravate the process of diabetic retinopathy by inducing nitrosative stress in the retinal neurovascular unit.nnnMETHODSnPeripheral blood mononuclear cell reactive oxygen species level was measured by flow cytometry along with spectrophotometric detection of malondialdehyde (MDA) and glutamate from serum or plasma and a vitreous sample of study groups (i.e. subjects with proliferative diabetic retinopathy [PDR], type 2 diabetes without retinopathy [DNR] and healthy controls [HCs]). Further, nitrosative stress assessment was performed by spectrophotometric and enzyme-linked immunosorbent assay-based detection of serum and vitreous nitrite and nitrotyrosine concentrations, respectively.nnnRESULTSnThe plasma glutamate level remains insignificant between subjects with PDR and DNR (p=0.505) or in HC (p=0.1344) individuals. However, serum MDA (p=0.0004), nitrite (p=0.0147) and nitrotyrosine (p=0.0129) were found to be strikingly higher among PDR subjects compared with the DNR group. Significantly increased levels of peripheral blood mononuclear cell reactive oxygen species (p<0.0001), vitreous glutamate (p=0.0009, p<0.0001), MDA (p=0.0058, p=0.0003), nitrite (p=0.0014, p<0.0001) and nitrotyrosine (p=0.0008, p<0.0001) were found in PDR subjects compared with DNR and HC subjects, respectively.nnnCONCLUSIONSnOur observation suggests that oxidative stress is associated with impairment in neuroretinal biochemical homeostasis among PDR subjects, which further augments retinal nitrosative stress and thus worsens the pathogenic process of retinopathy among PDR subjects.

Conservative management of severe bilateral emphysematous pyelonephritis: Case series and review of literature

Emphysematous pyelonephritis (EPN) is a life-threatening condition most commonly observed in diabetes, with nephrectomy believed to be the treatment of choice. However, nephrectomy in EPN is associated with increased risk of complications secondary to associated hemodynamic instability and may result in lifelong hemodialysis in case of bilateral EPN. We present three patients of severe bilateral EPN and one patient of unilateral EPN with diabetic ketoacidosis (DKA) successfully managed conservatively. Patient 1 (severe bilateral EPN) and patient 4 (unilateral EPN with DKA) responded to aggressive broad spectrum antibiotics, whereas patients 2 and 3 (severe bilateral EPN) responded to broad spectrum antibiotics along with percutaneous catheter drainage (PCD). PCD resulted in initial drainage of 300 and 200 ml of pus, respectively. All patients had associated uncontrolled hyperglycemia, poor glycemic control (HbA1c >8.5%), prerenal and intrinsic renal failure, leukocytosis, and dyselectrolytemia which responded to aggressive supportive management and insulin. There are several reports of successful medical management of severe bilateral EPN. Nephrectomy might no longer be the preferred treatment of severe bilateral EPN and may be reserved for patients’ refractory to antibiotics and PCD. Urgent randomized controlled trials are warranted in EPN to optimize the treatment protocols.

Occurrence of osteoporosis & factors determining bone mineral loss in young adults with Graves' disease

Background & objectives: There is a paucity of data with conflicting reports regarding the extent and pattern of bone mineral (BM) loss in Graves’ disease (GD), especially in young adults. Also, interpretation of BM data in Indians is limited by use of T-score cut-offs derived from Caucasians. This study was aimed to evaluate the occurrence of osteoporosis in active treatment naive patients with GD and determine the factors predicting BM loss, using standard T-scores from Caucasians and compare with the cut-offs proposed by the Indian Council of Medical Research (ICMR) for diagnosing osteoporosis in Indians. Methods: Patients with GD, >20 yr age without any history of use of anti-thyroid drugs, and normal controls without fracture history, drugs use or co-morbidities underwent BM density (BMD) assessment at lumbar spine, hip and forearm, thyroid function and calcium profile assessment. Women with menopause or premature ovarian insufficiency and men with androgen deficiency were excluded. Results: Patients with GD (n=31) had significantly lower BMD at spine (1.01±0.10 vs. 1.13±0.16 g/cm2), hip (0.88±0.10 vs. 1.04±0.19 g/cm2) and forearm (0.46±0.04 vs. 0.59±0.09 g/cm2) compared with controls (n=30) (P<0.001). Nine (29%) and six (19.3%) patients with GD had osteoporosis as per T-score and ICMR criteria, respectively. None of GD patients had osteoporosis at hip or spine as per ICMR criteria. Serum T3 had strongest inverse correlation with BMD at spine, hip and femur. Step-wise linear regression analysis after adjusting for age, BMI and vitamin D showed T3 to be the best predictor of reduced BMD at spine, hip and forearm, followed by phosphate at forearm and 48 h I131 uptake for spine BMD in GD. Interpretation & conclusions: Osteoporosis at hip or spine is not a major problem in GD and more commonly involves forearm. Diagnostic criterion developed from Caucasians tends to overdiagnose osteoporosis in Indians. T3 elevation and phosphate are important predictors of BMD. Baseline I131 uptake may have some role in predicting BMD.

Huge bilateral ovarian cysts in adulthood as the presenting feature of Van Wyk Grumbach syndrome due to chronic uncontrolled juvenile hypothyroidism.

Juvenile primary hypothyroidism causing cystic ovaries and pseudoprecocious puberty (Van-Wyk Grumbach syndrome (VWGS)) is well documented in literature. There are only a few reports of primary hypothyroidism presenting as ovarian cysts in adults. Here we present a case of huge bilateral ovarian cysts in adulthood as the presenting feature of VWGS due to chronic uncontrolled juvenile hypothyroidism. Large uniloculor right ovarian cyst (119 × 81 × 90 mm) and a multicystic left ovary (55 × 45 × 49 mm) were detected in a 24 year lady with secondary amenorrhea, galactorrhea, and palpable abdominal mass with history of neonatal jaundice, delayed milestones, short stature, and precocious menarche at age of 7.5 years age. She had elevated levels of cancer antigen (CA)-125 which normalized post levothyroxine supplementation. Elevated CA-125 may lead to misdiagnosis of ovarian carcinoma and inadvertent treatment. Bilateral ovarian cysts in adults are a rare presentation of juvenile hypothyroidism. It is necessary to screen for primary hypothyroidism in patients presenting with bilateral ovarian cysts to prevent unnecessary evaluation and treatment.

Ocular myasthenia with thyroid associated ophthalmopathy in subclinical Graves' disease: Diagnostic challenge and treatment outcomes

Ocular myasthenia in Graves′ disease is very rare with less than 10 cases reported. We present a clinically euthyroid lady with features of thyroid-associated ophthalmopathy (TAO) who had mild ptosis, external ophthalmoplegia, firm goiter and subclinical hyperthyroidism. Hertel exophthalmometry confirmed exophthalmos. CT orbit revealed thickening of bilateral inferior, medial and superior recti. Anti-thyroid peroxidase antibody was positive (154 IU/ml; normal <40 IU/ml). Repetitive nerve stimulation test of bilateral nasalis muscle showed decremental response, more prominent on the left side. Electrodiagnostic screening for concomitant myopathy or neuropathy was unremarkable. A subsequent positive neostigmine test and a positive anti-acetylcholine receptor antibody (1.46 nmol/L; normal <0.5 nmol/L) lead to the diagnosis of ocular myasthenia with TAO and subclinical Graves′ disease. Ptosis and ophthalmoplegia improved with pyridostigmine and carbimazole. She was doing well even after 2 years of follow-up. Any patient of TAO with ptosis and ophthalmoplegia should be evaluated for ocular myasthenia. This case intends to highlight that TAO and ocular myasthenia can coexist, is a diagnostic challenge, with important consequences, as lack of treatment of Graves′ disease can aggravate myasthenia.

Profile and Height Outcomes of Children with Short Stature in North India: An Experience from a Tertiary Care Centre

To the Editor: The ongoing dynamicity in the populationin terms of change in nutritional status, improved edu-cation, awareness and access to health care is expectedto bring changes in the etiologies causing short stature(SS). This study aimed to evaluate the current etiolog-ical trends for SS and the effect of treatment on finalheight. Retrospective analysis of records of childrenreferred for SS from2003–2007withatleast4yearsfollowup was done in the Department of Endocrinology,Vardhman Mahavir Medical College & Safdarjung Hos-pital, New Delhi. (Fig. 1). SS was defined as height<3rd percentile and/or height velocity <5th percentilefor chronological age.Records of 139 children [68 girls; 71 boys; age of pre-sentation: 11.34±3.71 y (Range 2–21 y)] were analyzed ofwhich74(53.24%)hadreached18yage.Thisstudyhighlights the predominantly late presentation of childrenwith SS in India. Familial short stature (FSS)(36, 26 %) wasthe most common cause of SS followed by hypothyroidism(26, 19 %), growth hormone deficiency (GHD) (19, 14 %),constitutional delay in growth and puberty (CDGP) (17,12 %), genetic syndromes [Turner’s(n=7); Down’ssyndrome (n=4)] (11, 8 %), Celiac disease (8, 6 %),nutritional (8, 6 %), achondroplasia/hypochondroplasia(6, 4 %), chronic disease (2, 1 %) and idiopathic(6,4 %). FSS and CDGP together constitute normalvariant SS (NVSS) and is the most common cause ofSS in our country, constituting 9 % [3], 16 % [4]and20 % [5] of all SS children in different studies and38 % in our study. This increased proportion of childrenwith NVSS in our series may reflect the increasedawareness in the society and increased healthcare seek-ing. Being a referral centre our patient profile is likelyto be skewed and it is likely that NVSS is even morecommon in the population.There was a significant difference in the presenting heightstandard deviation score (SDS) (P=0.002), final height SDS(P<0.001) and the change in height SDS (Δ Ht SDS)(P<0.001) depending on the etiology of SS (Table 1). Maxi-mum height improvement (ΔHt SDS) was seen in treatedGHD children followed by treated hypothyroid andCDGP. Poor height outcomeofchildrenwithCeliacdiseaseinourstudymaybetheresultofdelayeddiagnosisandlackofcompliance to gluten free diet. Poor height outcomes in chil-drenwithachondroplasia/hypochondroplasia and SS due tosyndromes reflect the limited growth potential in thesechildren. Endocrine disorders should be actively lookedfor, since they comprise of a third of children with SS andshowmaximumimprovementinheightontreatment.Thoughthere has been increased healthcare seeking,it is quite late asevident from our study. Increased awareness among generalpopulation and pediatricians would help early detection oftreatable cases and better height of the population.