Impact of Self-monitoring of Blood Glucose Log Reliability on Long-term Glycemic Outcomes in Children with Type 1 Diabetes.

INTRODUCTION: Logbooks of self-monitoring of blood glucose (SMBG) are useful in the modulation of insulin regimens, which aid in achieving glycemic control in type 1 diabetes mellitus (T1DM). However, discrepancies in SMBG charting may impede its utility. This study aimed to assess the accuracy of log entries and its impact on long-term glycemic control. METHODS: SMBG in logbooks was compared with readings in glucometer memory and discrepancies between the two were evaluated in 101 children with T1DM. The relationship between these discrepancies and glycated hemoglobin (HbA1c) over 44 months was assessed. RESULTS: Errors in glucose charting were observed in 32.67% children. The most common observed error was omission (42.42%), followed by fabrication (27.27%), erroneous (18.18%), and others (12.12%). Age was not significantly different among children having accurate versus inaccurate SMBG logs. During follow-up of 44 months, children with accurate SMBG logs consistently had lower HbA1c as compared to children having inaccurate logs, which was statistically significant at 4, 16, 20, and 28 months' follow-up. The same was reflected in the proportion of children achieving HbA1c <7% and 7%-9%. Of the 14 children who had omissions, 9 had omission of high values only, 3 patients had omission of low values only, 1 had omission of both high and low values, and 1 had omission of normal values. Among logs with fabrication, parents were responsible in 2 of 9 incidents. In the remaining 7, it was the child himself/herself. Children with fabrication consistently had the highest HbA1c values among the different types of inaccurate blood glucose chartings, which was statistically significant at 32 and 36 months of follow-up. CONCLUSIONS: Reliability of SMBG logs is a significant problem among children with T1DM at our center. Children with accurate logs of SMBG readings were more likely to have better glycemic control on long-term follow-up.

Effects of thyroid status on glycated hemoglobin.

INTRODUCTION: Glycated hemoglobin (HbA1c) can be altered in different conditions. We hypothesize that HbA1c levels may change due to altered thyroid status, possibly due to changes in red blood cell (RBC) turnover. OBJECTIVES: The objective of this study was to determine the effects of altered thyroid status on HbA1c levels in individuals without diabetes, with overt hyper- and hypo-thyroidism, and if present, whether such changes in HbA1c are reversed after achieving euthyroid state. METHODS: Euglycemic individuals with overt hypo- or hyper-thyroidism were selected. Age- and sex-matched controls were recruited. Baseline HbA1c and reticulocyte counts (for estimation of RBC turnover) were estimated in all the patients and compared. Thereafter, stable euthyroidism was achieved in a randomly selected subgroup and HbA1c and reticulocyte count was reassessed. HbA1c values and reticulocyte counts were compared with baseline in both the groups. RESULTS: Hb A1c in patients initially selected was found to be significantly higher in hypothyroid group. HbA1c values in hyperthyroid patients were not significantly different from controls. HbA1c reduction and rise in reticulocyte count were significant in hypothyroid group following treatment without significant change in glucose level. Hb A1c did not change significantly following treatment in hyperthyroid group. The reticulocyte count, however, decreased significantly. CONCLUSION: Baseline HbA1c levels were found to be significantly higher in hypothyroid patients, which reduced significantly after achievement of euthyroidism without any change in glucose levels. Significant baseline or posttreatment change was not observed in hyperthyroid patients. Our study suggests that we should be cautious while interpreting HbA1c data in patients with hypothyroidism.

Neck height ratio is an important predictor of metabolic syndrome among Asian Indians.

BACKGROUND AND AIMS: The predictive potential of neck circumference (NC) based indices (a measure of upper body fat distribution) for predicting metabolic syndrome (MetS) and its components among Indians is not known. This study aimed to evaluate the role of NC and neck height ratio (NHtR) as independent predictors of MetS and its components as compared to traditional anthropometric indices. MATERIALS AND METHODS: A total of 451 individuals from 867 screened individuals, 30-80 years age, without any co-morbid state who gave informed written consent underwent clinical, anthropometric, and biochemical assessment. RESULTS: Patients with MetS in both the sexes had significantly higher NC, NHtR, glycated hemoglobin, fasting glucose, and dyslipidemia (higher triglycerides, total cholesterol/high-density lipoprotein cholesterol (HDL-C) ratio, low-density lipoprotein cholesterol/HDL-C ratio, and lower HDL-C). In both sexes, individuals in the highest tertile of NC had significantly greater central and generalized obesity, lower HDL-C, and significantly higher MetS. Receiver operating characteristic analysis revealed waist circumference (WC) to have the largest area under the curve for predicting MetS in both sexes, followed by NHtR, NC, and body mass index. NC and NHtR of >34.9 cm (sensitivity 78.6%; specificity 59.3%) and >21.17 cm/m (sensitivity 80.7% and specificity 64.6%) respectively for men and >31.25 cm (sensitivity 72.3%; specificity 64.4%) and >20.48 cm/m (sensitivity 80.4% and specificity 60%) respectively for women were the best values for identifying MetS. Increased NC and NHtR had odds ratio of 1.52 (95% confidence interval [CI]: 1.37-1.68; P < 0.001) and 1.96 (95% CI: 1.67-2.29; P < 0.001) respectively in identifying MetS. CONCLUSION: NC and NHtR are good predictors of MetS and cardiovascular risk factors in Asian Indians. NHtR is reliable and perhaps an even better index than NC with regards to cardiovascular risk prediction.

Precocious puberty: A blessing in disguise!

Germ cell tumors may lead to incomplete isosexual male precocity and are commonly located in the pineal gland. Germinomas of the basal ganglia are almost always unilateral and precocious puberty is a rare manifestation in them. We report a 9.5-year-old boy who presented with incomplete isosexual precocity due to bilateral basal ganglia germinoma.

A rare case report of 46XY mixed gonadal dysgenesis.

A 16-year-old person, reared as female presented with complaints of genital ambiguity and primary amenorrhoea along with lack of secondary sexual characters, but without short stature and Turner's stigmata. She was taking steroids after being misdiagnosed as congenital adrenal hyperplasia (CAH). Karyotype analysis revealed 46XY karyotype. There was no evidence of hypocortisolemia (cortisol 9.08 µg/dl, adrenocorticotropic hormone [ACTH] 82.5 pg/ml) or elevated level of 17-OH-progesterone (0.16 ng/ml). Pooled luteinizing hormone (LH) was 11.79 mIU/ml and follicle-stimulating hormone (FSH) was 66.37 mIU/ml. Serum estradiol level was 25 pg/ml (21-251). Basal and 72 h post beta-human chorionic gonadotropin (hCG) levels of androstenedione and testosterone levels were done (basal testosterone of 652 ng/dl and basal androstenedione of 1.17 ng/ml; 72 h post hCG testosterone of 896 ng/dl and androstenedione of 1.34 ng/ml). Magnetic resonance imaging (MRI) pelvis (with ultrasonogrphy [USG] correlation) revealed uterus didelphys with obstructed right moiety and bilateral ovarian-like structures. Right sided gonads and adjacent tubal structures were visualized laparoscopically and removed. Left sided gonads were not visualized and Mullerian remnants were adhered to sigmoid colon. Histopathological examination revealed presence of testicular tissue showing atrophic seminiferous tubules with hyperplasia of Leydig cells. No ovarian tissue was seen. Based on these results a diagnosis of 46XY mixed gonadal dysgenesis (MGD) was made, which is rare and is difficult to distinguish from 46XY ovotesticular disorder of sexual differentiation (OT-DSD). The patient was managed with a multidisciplinary approach and fertility issues discussed with the patient's caregivers.

Medical management of thyroid ectopia:report of three cases.

Thyroid ectopia (TE) is an embryological aberration of the thyroid gland migration most commonly observed in the lingual region followed by the sublingual, hyoid, and mediastinal regions. TE is often complicated by local compressive symptoms resulting in dysphagia, dysphonia, and dyspnea. Surgical removal of TE is frequently complicated by difficulties in intubation, increased perioperative bleeding, and severe primary hypothyroidism; on the other hand, I131 ablation is limited by high doses needed and the concern for long-term effects especially in children. We report three children with TE who all presented with compressive symptoms and were managed conservatively with levothyroxine resulting in resolution of compressive symptoms and favorable outcomes. Levothyroxine supplementation is effective and has an important role in managing TE, not only in correcting the associated hypothyroidism but also in resolving the associated compressive symptoms by reducing the size of the ectopic thyroid tissue.

Thyroid associated orbitopathy with ocular myasthenia in primary hypothyroidism: Keep those eyes open.

Thyroid associated orbitopathy, although seen most commonly with thyrotoxicosis, is also known to occur in primary hypothyroidism. Myasthenia gravis is an autoimmune condition with an established association with autoimmune thyroid disease. We report the case of a patient who presented with recent onset unilateral ptosis that was fatigable with a history of proptosis since a year. On examination, she had a goiter, bilateral proptosis, restriction of upward gaze and adduction both eyes and normal pupils. Investigations revealed primary hypothyroidism with anti-thyroid peroxidase positive and anti-acetylcholine receptor antibody positive. Computerized tomography orbit showed thickening of medial and inferior rectus characteristic of thyroid orbitopathy. A diagnosis of primary hypothyroidism with thyroid orbitopathy with ocular myasthenia gravis was made. Patient is on Levothyroxine and anticholinesterase medications and is on follow-up. We present this case to highlight that the presence of ptosis in a patient with thyroid orbitopathy should alert the clinician to the possible coexistence of myasthenia gravis.

Vitamin D status of patients with type 2 diabetes and sputum positive pulmonary tuberculosis.

INTRODUCTION: Vitamin D deficiency is expected to be higher in patients with diabetes and pulmonary tuberculosis (TB). Studies estimating prevalence in the subset of patients with both diabetes and pulmonary TB are scarce. MATERIALS AND METHODS: A total of 155 subjects were recruited; 46 patients with type 2 diabetes, 39 non-diabetic healthy controls, 30 patients of pulmonary TB and 40 patients with both pulmonary TB and type 2 diabetes. Vitamin D level (25 OH vitamin D) levels were done for all the 4 groups. RESULTS: Mean vitamin D levels were not different between groups with TB, diabetes mellitus or combination of both, but the prevalence of severe vitamin D deficiency was higher in the group with both diabetes and TB (45%) as compared with the group with only TB (26.66%) and diabetes (17.39%) and healthy controls (7.69%). CONCLUSION: The prevalence of patients with severe vitamin D deficiency is higher in patients with dual affection of TB and diabetes mellitus as compared with either disorder alone implying that patients with type 2 diabetes with the most severe vitamin D deficiency are the one of the most predisposed to pulmonary TB.

Atypical neurofibroma and osteosclerotic metastasis.

35-year-old male presented with multiple swellings in left leg, headache, weakness of limbs for 4 months, and blurring of vision for the last 15 days. On examination, he was pale, cachexic with generalized lymphadenopathy and lower motor neuron type weakness of limbs sparing right upper limb. Blood investigations showed anemia with high alkaline phosphatase. Chest radiograph revealed osteosclerotic metastatic lesion in humerus. Biopsy of leg lesion revealed atypical neurofibroma. Computed tomography (CT) of thorax revealed osteoblastic metastasis. Bone marrow aspiration showed cells with round to oval nuclei, fine granular chromatin with large central prominent nucleoli and eosinophilic cytoplasm with acini formation. Magnetic resonance imaging (MRI) of brain and spinal cord defined metastatic leptomeningeal deposits. Cerebrospinal fluid (CSF) cytology was positive for malignant cells. Gastroscopy showed an ulceroinfiltrative growth from stomach which on histopathology revealed diffuse adenocarcinoma. Palliative treatment was given with intrathecal methotrexate and systemic corticosteroid with chemotherapy. Patient's symptom improved drastically, but we lost him to followup.

A rare case of Balo concentric sclerosis showing unusual clinical improvement and response with oral prednisolone.

The present report is a rare case of Balo Concentric Sclerosis. Most cases have either been diagnosed post mortem or have succumbed to the disease after being diagnosed ante mortem. In our case, the patient showed a dramatic response to treatment, and after a one-year follow-up, he was asymptomatic, with no relapses or residual effect of the illness.

The "Double A" phenotype: Portending Allgrove's syndrome and averting adrenal crisis.

INTRODUCTION: Allgrove's syndrome is a rare autosomal-recessive disorder with only about 70 cases reported thus far and is characterized by alacrima, achalasia, and ACTH insensitivity among other clinical features. However, it has a widely variable clinical presentation, which may result in such cases remaining undiagnosed. OBJECTIVE: To report a patient with impending Allgrove's syndrome and to highlight the importance of clinical suspicion in diagnosing the same. MATERIALS AND METHODS: A 2.5-year-old girl was diagnosed with impending Allgrove's syndrome on the basis of clinical presentation, barium swallow study, Schirmer's test, and hormonal evaluation. RESULTS: A 2.5-year-old girl, born of non-consanguineous marriage, presented with failure to thrive and developmental delay with occasional vomiting on taking solid or semi-solid food for past 6 months. Examination revealed stunted weight (SDS of -4.4) and height (SDS of -4.76), and barium swallow showed presence of achalasia. On direct questioning, her mother mentioned presence of decreased tears on crying since birth, and Schirmer's test confirmed the presence of dry eyes. Baseline ACTH was slightly elevated with normal basal and post-ACTH stimulation serum cortisol. Based on these findings, impending Allgrove's syndrome was diagnosed with a plan for follow-up study of adrenal function. CONCLUSIONS: Allgrove's syndrome may be an under diagnosed disorder as aclarima is often overlooked. However, a high index of clinical suspicion may help in avoiding adrenal crisis by diagnosing the condition early.

Evolving adrenal insufficiency.

INTRODUCTION: Tuberculosis is the most common cause of Addison's disease in India. The exact status of adrenal reserve in tuberculosis is still an enigma and recovery of adrenal function is unpredictable. OBJECTIVE: We report a case with a pre-Addisonian state and unchanged adrenal size after 1 year treatment. MATERIALS AND METHODS: A 31-year patient with adrenal tuberculosis was diagnosed and treated with anti tubercular drugs (ATDs) and steroid. RESULTS: A 31-year male, presented with fever and weight loss for 3½ months with anorexia, nausea, hyperpigmentation of skin, and buccal mucosa and weakness with past h/o adequately treated pulmonary tuberculosis at 3 years of age. On examination, the patient was anemic. A non-tender, firm right (Rt.) submandibular lymphnode was palpable. Investigations revealed: High erythrocyte sedimentation rate (ESR), negative HIV, and sputum for acid fast bacilli (AFB). Initial cortisol was high but subsequently became low with negative short synacthin test (SST). Computed tomography showed bilateral (B/L) enlarged hypodense adrenal mass with inconclusive fine needle aspiration cytology (FNAC) and negative AFB culture. Rt. submandibular lymph node FNAC showed caseating granuloma. ATDs and steroids were started, the lymphadenopathy regressed and symptoms subsided. However, after 1 year of treatment steroid withdrawal failed and adrenal size remained the same. CONCLUSION: The adrenal has considerable capacity to regenerate during active infection and ultimately become normal or smaller in size. However, in the case reported here, they failed to regress. Reversal of adrenal function following ATD is a controversial issue. Some studies have shown normalization following therapy, while others have contradicted it similar to the finding in our case.

Refractory rickets due to Fanconi's Syndrome secondary to Wilson's disease.

Renal tubular disorders are an important cause of refractory rickets. Wilson's disease, an inherited disorder of copper metabolism has varied presentations. We present a case of refractory rickets due to Fanconi's syndrome attributable to Wilson's disease. An adolescent girl presented with pain in the hip and knee joints and a knock-knee deformity since six years. She had received multiple doses of cholecalciferol with little improvement. There was no history of seizures, polyuria, jaundice, intake of drugs, or similar complaints in the family. Examination revealed a severely short stature with widening of the wrist joint and genu valgum. Examination of the central nervous system (CNS) was normal. Skeletal radiographs showed features suggestive of rickets at the hip and knee joints. Routine biochemistry was normal, 25-hydroxyvitamin D [25(OH)D] was adequate (57.1 ng/dL), with normal corrected calcium (9.24 mg/dL), low phosphate (2.76 mg/dL), elevated bone-specific alkaline phosphatase, and normal renal functions. Twenty-four-hour urine revealed phosphaturia, kaliuresis, and glucosuria with normal blood sugars and aminoaciduria. Blood gas analysis revealed normal anion gap metabolic acidosis with a urine pH of 7. Ammonium chloride (NH4CL) challenge test revealed proximal tubular acidosis. A search for causes revealed Kayser-Fleischer rings. The diagnosis of Wilson's disease was confirmed by low serum ceruloplasmin levels (6.5 mg/dL; normal: 18-35 mg/dL) with high 24-hour urine copper levels (433 mcg; normal: 20-50 mcg). She was started on a replacement of alkali, phosphate, calcium, and vitamin D, with zinc acetate for Wilson's disease. Rickets as a presenting feature of Wilson's disease has been reported rarely. Recognition of this entity is important, as treatment of the primary condition may improve tubular function as well.

De morseir syndrome presenting as ambiguous genitalia.

BACKGROUND: A 10-year-old boy presented with genital ambiguity, poor linear growth, and delayed milestones. The aim and to highlight that although rare but congenital, hypogonadotropic hypogonadism may rarely present as ambiguity. MATERIALS AND METHODS: The patient was found to have bilateral cryptorchidism with proximal penile hypospadias, microphallus with a proportionate dwarfism with mildly delayed bone age, and karyotype 46XY. Euthyroid with normal steroid axis, growth hormone insufficient as suggested by auxology, low IGF1, and poor response to clonidine stimulation. MRI brain shows hypoplastic corpus callosum, hypoplastic anterior pituitary, and ectopic posterior pituitary bright spot. RESULTS: The patient underwent laparoscopic removal of right intrabdominal testis and orchidoplexy was performed on the left one. Testicular biopsy revealed no malignancy and growth hormone replacement was initiated. The patient awaits definitive repair of hypospadias. CONCLUSION: As a provisional diagnosis of combined growth hormone and gonadotropin deficiency, most probable diagnosis is septo-optic dysplasia or de moseir syndrome leading to genital ambiguity.