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1.
Objective
To evaluate the frequency of non-autoimmune subclinical and overt hypothyroidism in children with idiopathic steroid-resistant nephrotic syndrome (SRNS).Methods
This cross-sectional study recruited 30 children (age 1–18 y) with idiopathic SRNS; and 30 healthy controls. Serum T3, T4 and TSH were performed in cases as well as controls. Anti-thyroid peroxidase and anti-thyroglobulin antibody tests were performed in all cases.Results
Non-autoimmune subclinical or overt hypothyroidism was detected in 10 out of 30 children with idiopathic SRNS; 2 had overt hypothyroidism, while 8 patients had subclinical hypothyroidism. Children with SRNS had a mean (SD) TSH value 4.55 (4.64) mIU/L that was higher as compared to controls (1.88 (1.04) mIU/L) (P<0.01). Focal segmental glomerulosclerosis (FSGS) was the commonest histopathological condition, seen in 13 (43.3%). Children with overt hypothyroidism (2 cases) and grade III subclinical hypothyroidism (1 case) were subsequently started on levothyroxine therapy.Conclusions
The prevalence of subclinical and overt hypothyroidism seems to be high in idiopathic SRNS, with almost one-third of children having overt or subclinical non-autoimmune hypothyroidism.2.
Maria Scavone Elena Carboni Ettore Stefanelli Giusy Romano Anna Vero Laura Giancotti Roberto Miniero Valentina Talarico 《Indian pediatrics》2018,55(12):1059-1061
Objective
To identify factors that discriminate between transient and permanent congenital hypothyroidism.Methods
Retrospective evaluation of 58 children with congenital hypothyroidism and eutopic thyroid gland. Gender, gestational age, birth weight, TSH and serum thyroxine levels at diagnosis and L-thyroxine dose at 12 and 24 months of age were analyzed.Results
Median (IQR) initial TSH levels were 73.3 (276.5) μIU/mL in permanent hypothyroidism and 24.24 (52.7) μU/mL in transient hypothyroidism (P =0.0132). The optimum cut-off value of initial TSH to predict transient hypothyroidism was 90 μIU/mL. Mean (SD) L-thyroxine doses at 24 months of age were 2.64 (0.98) μg/kg/day in permanent hypothyroidism and 1.91 (0.65) μg/kg/day in transient hypothyroidism. Requirement of Lthyroxine dose at 24 months of ≤0.94 μg/kg/day had the highest sensitivity (100%) to predict transient hypothyroidism.Conclusions
L-thyroxine doses at 24 months can predict transient hypothyroidism in patients with eutopic thyroid gland earlier than at 36 months.3.
Mathrubootham Sridhar Shriraam Mahadevan Latha Vishwanathan Anbezhil Subbarayan 《Indian pediatrics》2018,55(3):219-221
Objective
To assess the natural history and progression of subclinical hypothyroidism and to study factors which help predict evolution of subclinical hypothyroidism into overt hypothyroidism.Methods
Longitudinal study in 40 children (2-16 yrs) presenting with subclinical hypothyroidism in a tertiary care unit in Chennai, India. Patients showing evidence of overt hypothyroidism or thyroid stimulating hormone ≥15 mIU/mL during follow-up were started on thyroxine. Others were followed up with 3-monthly thyroid function tests up to one year.Results
At the end of our study period 3 (7.5%) were overtly hypothyroid, 16 (40%) remained as subclinical hypothyroid, and 21 (52.5%) became euthyroid. Evidence of autoimmunity at baseline was a significant (P<0.05) risk factor for progression to overt hypothyroidism.Conclusions
Subclinical hypothyroidism in children, with thyroid stimulating hormone upto 15 mIU/L and irrespective of thyroid autoimmunity, needs only periodic clinical and biochemical follow up. Thyroid autoimmunity may point to an increased probability of progression to overt hypothyroidism.4.
S. Mehar Sulthana S. Nandha Kumar M. G. Sridhar B. Vishnu Bhat K. Ramachandra Rao 《Indian journal of pediatrics》2012,79(11):1473-1476
Objectives
To confirm the clinical diagnosis of Down syndrome by chromosomal analysis and to explore the oxidative stress in children with Down syndrome by estimating the levels of non enzymatic antioxidants like reduced glutathione(GSH) and total antioxidants status (TAS).Methods
The study included 31 clinically diagnosed children with Down syndrome with equal number of age and sex matched controls. Trisomy 21 was confirmed by conventional lymphocyte cell culture. Erythrocytic reduced glutathione (GSH) and plasma total antioxidant status (TAS) were measured sphectrophotometrically.Results
The levels of erythrocytic reduced glutathione (GSH) and plasma total antioxidant status (TAS) were significantly reduced in children with Down syndrome.Conclusions
Children with Down syndrome have elevated levels of oxidative stress . Hence antioxidant therapy can be beneficial among them.5.
Background
Disorders of the thyroid function of mother and child can not only irreversibly inhibit maturation of the central nervous system, but may also affect growth and puberty.Objectives
Review of the consequences of thyroid function disorders on the growth of children and adolescents and the relevance of adequate treatment.Materials and Methods
This article summarises the current literature concerning the effect of thyroid hormone disorders of mother and child and their therapy on growth.Results
Every untreated manifestation of congenital or acquired hypothyroidism is associated with a delay of growth and skeletal maturation. In children presenting with acquired hyperthyroidism, height is shifted towards higher values and skeletal maturation is accelerated. Approximately 20?% of children and adolescents with Down syndrome develop primary hypothyroidism, frequently manifesting within the first months of life (approximately 5.5?%). Adequate treatment of the thyroid disorder can normalise growth disorders. About 6?% of children with growth hormone deficiency develop abnormally low free thyroxin (fT4) levels in serum during growth hormone treatment due to increased conversion of T4 to T3. There is no indication for treatment after excluding secondary hypothyroidism.Conclusions
Early diagnosis and timely, adequate treatment of thyroid function disorders of mother and child are essential to achieving growth that is as age-appropriate as possible in affected children and adolescents6.
Khaled Rahmani Shahin Yarahmadi Koorosh Etemad Yadollah Mehrabi Nasrin Aghang Ahmad Koosha Hamid Soori 《Indian pediatrics》2018,55(2):121-124
Objective
To evaluate the success rate of the National newborn screening program in maintenance of intelligent quotient (IQ) of children with congenital hypothyroidism in Iran.Design
Retrospective cohort study.Methods
The IQ scores, in three subsets of verbal, non verbal (Performance) and full scale IQ, of 240 children diagnosed with Transient congenital hypothyroidism (TCH) and Permanent congenital hypothyroidism (PCH), from 5 provinces in 5 different geographical areas of Iran, were measured at the age of 6 years using revised Wechsler pre school and primary scale of intelligence and compared with 240 healthy children. We used independent sample t test and two-way ANOVA for data analysis.Results
Mean of verbal, performance, and full scale IQ scores were lower in the CH cases (permanent and transient) than the control group. Most of the IQ differences in two studied groups related to the PCH cases (P=0.005). Mean difference of IQs between children in the two groups in Yazd province (center of Iran) was higher than other provinces, and they also had significantly lower IQ than their control (healthy) children (P=0.001). No treated child had IQ<70.Conclusion
Although mean IQs of CH children was lower than their controls, IQ of all treated CH cases were close to the healthy children.7.
Lakshminarasimhan Sindhuja Devi Dayal Kushaljit Singh Sodhi Naresh Sachdeva Anish Bhattacharya 《World journal of pediatrics : WJP》2016,12(2):215-218
Background
Familial clustering in patients with permanent congenital hypothyroidism (CH) caused by thyroid dysgenesis (TD) has been reported in developed countries. There is no information on familial TD from developing countries.Methods
A total of 312 first degree relatives belonging to 80 families of children with TD (group 1) and 40 families of age-matched normal children (group 2) were screened by thyroid ultrasonography, serum total thyroxine (T4) and thyroid stimulating hormone (TSH).Results
Thyroid scintigraphy revealed agenesis in 78.7% of the patients, ectopic gland in 15%, and hypoplasia in 6.2%. The mean thyroid volumes were similar in parents and siblings of both groups. Eight (10.6%) mothers in group 1 were identified to have thyroid hypoplasia as compared with none in group 2 (P=0.03). Serum TSH was significantly higher in group 1 than in group 2 (P=0.004). Sixteen (7.8%) subjects (6 mothers, 5 fathers, and 5 siblings) in group 1 were found to have subclinical hypothyroidism as compared to none in group 2 (P<0.05). Four families were identified to have thyroid developmental anomalies and abnormal thyroid functions accounting for 5% of cases of familial TD in our cohort.Conclusion
Thyroid developmental anomalies and thyroid function abnormalities are more frequent in first degree relatives of children with TD as compared with a control population. These findings suggest that possibly there is a genetic component of TD in Indian patients.8.
Background
Nephrotic syndrome is a rare but severe feature of IgA nephropathy.Case characteristics
Nine Japanese children with severe IgA nephropathy with nephrotic syndrome.Intervention
All received low-dose intravenous methylprednisolone (IVMP) within five weeks after the disease onset. Eight out of nine patients achieved resolution of proteinuria without severe adverse events.Message
Early low-dose intravenous methylprednisolone may be safe and effective for children with severe IgA nephropathy with nephrotic syndrome.9.
Background
The clinical management of intravenous immunoglobulin-resistant Kawasaki disease shock syndrome (KDSS) is obscure.Case characteristics
Three children presented with intravenous immunoglobulin-resistant KDSS complicated with myocarditis.Outcome
All cases were successfully managed with steroid pulse therapy.Message
Steroid pulse therapy is effective in immunoglobulin-resistant KDSS.10.
Background
Antiphospholipid syndrome is a multisystem auto-immune disorder characterized by arterial or venous thrombosis in children.Case characteristics
11-year-old child with pneumococcal meningitis also had cerebral sinus vein thrombosis and pulmonary artery segmental thrombosis.Observation
Pro-thrombotic evaluation showed positive lupus anticoagulant at baseline and after 12 weeks. Investigations for lupus were negative at admission and after one year of follow-up.Message
Antiphospholipid syndrome is a possibility even in thrombosis occurring in the setting of meningitis.11.
Bahar Toklu Baysal Bora Baysal Ferah Genel Baris Erdur Erhan Ozbek Korcan Demir Behzat Ozkan 《Indian pediatrics》2017,54(5):381-384
Objective
To study the factors affecting a neurodevelopmental status of children with congenital hypothyroidism, diagnosed on national screening program.Methods
The study was performed in the Pediatric Endocrinology Department of Dr. Behcet Uz Children’s Hospital between May 2012 and May 2013. Children with congenital hypothyroidism, aged between 24 and 36 months, diagnosed by national screening program were included in the study group. Healthy subjects at the same age group consisted of the control group. For the neurodevelopmental evaluation, Bayley Scale of Infant Development- II (BSID-II) was used. Factors possibly effective on neurodevelopment were evaluated.Results
42 patients and 40 healthy children (mean (SD) age, 29.4 (3.7) and 29.2 (3.5), respectively were included in the study. The mean MDI score [92.6 (7.07) vs 97.1 (9.69), P=0.14)] and the mean PDI score [97.8 (15.68) vs 99.1 (10.57), P=0.66)] in the study group and control group were not significantly different. Among the patient, 4.6% and 4.7% children were moderately retarded as per the MDI scores and PPI scores, respectively. The sex, socioeconomic status, birth weight, screening levels of TSH, severity of the congenital hypothyroidism, initiation time and the dosage of thyroid hormone replacement, length of the normalization period of TSH, and adherence to treatment were not found to affect the MDI and PDI scores of the patients.Conclusion
Some children with congenital hypothyrodism may have mild to moderate neurodevelopmental retardation, despite the early diagnosis and treatment, and thus need to be under regular follow-up for neurodevelopmental status.12.
Manoja Kumar Das Vidyut Bhatia Anupam Sibal Abha Gupta Sarath Gopalan Raman Sardana Reeti Sahni Ankur Roy Narendra K Arora 《Indian pediatrics》2017,54(12):1012-1016
Objective
To document the prevalence of non-alcoholic fatty liver disease (NAFLD) and metabolic parameters among normalweight and overweight schoolchildren.Design
Cross-sectional study.Setting
Thirteen private schools in urban Faridabad, Haryana.Participants
961 school children aged 5-10 years.Methods
Ultrasound testing was done, and 215 with fatty liver on ultrasound underwent further clinical, biochemical and virological testing.Outcome measures
Prevalence of fatty liver on ultrasound, and NAFLD and its association with biochemical abnormalities and demographic risk factors.Results
On ultrasound, 215 (22.4%) children had fatty liver; 18.9% in normal-weight and 45.6% in overweight category. Presence and severity of fatty liver disease increased with body mass index (BMI) and age. Among the children with NAFLD, elevated SGOT and SGPT was observed in 21.5% and 10.4% children, respectively. Liver enzyme derangement was significantly higher in overweight children (27% vs 19.4% in normal-weight) and severity of fatty liver (28% vs 20% in mild fatty liver cases). Eleven (8.1%) children with NAFLD had metabolic syndrome. Higher BMI (OR 35.9), severe fatty liver disease (OR 1.7) and female sex (OR 1.9) had strong association with metabolic syndrome.Conclusions
22.4% of normal-weight and overweight children aged 5-10 years had fatty liver. A high proportion (18.9%) of normal-weight children with fatty liver on ultrasound indicates the silent burden in the population.13.
Background
A recent randomized control trial in children with steroid-resistant nephrotic syndrome revealed that two doses of rituximab did not reduce proteinuria.Case characteristics
A 14-month-old boy developed refractory steroid-resistant nephrotic syndrome due to focal segmental glomerulosclerosis.Observation
The patient achieved complete remission 11 months after disease onset following eight doses of rituximab combined with steroids and cyclosporine.Message
Long-lasting B cell depletion with repeated rituximab administrations may be required to achieve complete remission in patients with steroid-resistant nephrotic syndrome and massive proteinuria.14.
AT Kaaviyaa Vidya Krishna TS Arunprasath Padmasani Venkat Ramanan 《Indian pediatrics》2018,55(11):969-971
Objective
To study the association between asthma control and serum 25OH Vitamin D levels in children with moderate persistent asthma on preventer therapy.Methods
Children aged 6–18 years, with moderate persistent asthma, on preventer therapy for ≥2 months were included. Control was categorized as good, partial or poor as per GINA guidelines. Serum 25 (OH) Vitamin D levels were measured and their relationship with the level of control was studied.Results
Out of 50 children enrolled, 22 had well-controlled asthma, and 21 had partially controlled asthma. Vitamin D was deficient in 30 children and insufficient in 18 children. Children with vitamin D deficiency had significantly less wellcontrolled asthma as compared to those with insufficient or sufficient levels of 25 (OH) vitamin D (13.3% vs 88.9 % vs 100%).Conclusion
Vitamin D deficiency is associated with suboptimal asthma control.15.
Vikrant Sood Rajeev Khanna Dinesh Rawat Shvetank Sharma Seema Alam Shiv Kumar Sarin 《Indian pediatrics》2018,55(7):561-567
Objectives
To find association of pediatric NAFLD with metabolic risk factors, and Patatin-like phospholipase domain-containing protein 3 (PNPLA3) gene polymorphism.Design
Cross-sectional studySetting
Pediatric Hepatology unit of a tertiary care hospitalParticipants
Overweight/obese children (<18 years) with (69 patients) or without (30 patients) NAFLD (ultrasonography based), and their parents.Intervention
Metabolic screening, PNPLA3 gene polymorphism, and transient elastographyOutcome measure
Association of pediatric NAFLD with parental metabolic risk factors and PNPLA3 gene polymorphism.Results
In the NAFLD group, there was high parental incidence of metabolic diseases, fatty liver (80%) and low high-density lipoproteins levels (84%). Family history of NAFLD (in any parent), higher alanine aminotransferase levels and higher total cholesterol levels in the child independently predicted possibility of NAFLD, but similar results could not be replicated for PNPLA3 gene polymorphism. Controlled attenuation parameter measurement (by transient elastography) had high sensitivity and specificity to diagnose steatosis.Conclusion
There is high familial incidence of metabolic diseases in children with NAFLD. Controlled attenuation parameter can be useful as a non-invasive modality to screen fatty liver in children.16.
Meriem Zahed-Cheikh Barthélémy Tosello Stéphanie Coze Catherine Gire 《Indian pediatrics》2017,54(6):495-497
Background
The kyphoscoliotic type of Ehlers-Danlos syndrome (EDS type VIA) is an autosomal recessive disorder characterized by connective tissue dysplasia.Case characteristics
We report two children with perinatal stroke; accompanied by neonatal joint hypermobility, hypotonia; and early development of kyphoscoliosis.Outcome
Molecular analysis revealed a PLOD1 gene mutation. Our definitive diagnosis was a EDS VIA.Message
Prenatal brain stroke is a rare clinical feature of EDSVIA.17.
Background
Synovial arteriovenous malformation is rare.Case characteristics
We present three children with recurrent monoarthritis secondary to synovial arteriovenous malformation.Outcome
Two children underwent excision of arteriovenous malformation. Another child had diffuse arteriovenous malformation, which was inoperable.Message
Synovial arteriovenous malformations should be considered in the differential diagnosis of monoarthritis, especially of the knee.18.
Rajeev Redkar Anant Bangar Varun Hathiramani Vinod Raj C. Swathi 《Indian pediatrics》2018,55(10):871-873
Objective
To study the diagnostic methods and treatment outcomes in children with Budd-Chiari syndrome.Methods
Case records of 25 patients with Budd-Chiari syndrome were evaluated retrospectively. These patients were investigated with imaging techniques and underwent balloon angioplasty or surgical management.Results
21 patients underwent balloon angioplasty, of which 17 had good medium- to long-term results, while only one out of four patients who underwent a portocaval shunt survived.Conclusion
The balloon angioplasty has satisfactory outcome in the treatment of acute Budd-Chiari syndrome. In failed cases, the surgical therapy may be attempted, but the outcomes do not appear rewarding.19.
Background
Blue rubber bleb nevus syndrome is a rare disease involving venous malformations.Case characteristics
We present a 6-year-old female with the syndrome, and consumptive coagulopathy.Intervention/Outcome
After management with sirolimus, symptoms resolved.Message
Sirolimus may be a valuable option for reducing bleeding complications and cosmetic sequelae for the patients with this syndrome.20.