Harish Pemde - Academia.edu (original) (raw)
Papers by Harish Pemde
PubMed, May 14, 2023
Justification: The transgender community has been long stigmatized, and discriminated against, an... more Justification: The transgender community has been long stigmatized, and discriminated against, and faces numerous mental and physical problems. Certain indicators of transgender personality appear during childhood and more often before puberty begins. This puts the onus on Pediatricians to identify and offer evidence-based care for their benefit. There is an urgent and deep-felt need to understand the medical, legal, and social aspects of the care of transgender children. Hence, Adolescent Health Academy decided to release a statement on the care of transgender children, adolescents, and youth. Objectives: To review the existing international and national guidelines and recommendations to formulate a statement for the Pediatricians on (a) terminologies and definitions; (b) legal status in India; and (c) implications for pediatric practice. Process: A task force was convened by the Adolescent Health Academy as the writing committee to draft the guidelines. These were approved by all the members of the task force and the Executive Board of Adolescent Health Academy (2022). Recommendations: Gender identity develops in childhood and adolescence as a feeling of self, and it should be respected to mitigate gender dysphoria. The law permits transgenders the right of self-affirmation and it upholds their dignity in society. The transgender community is prone to victimization, and prejudice leading to a high risk of substance abuse, suicidal ideation, and mental health issues. Pediatricians are the primary care providers of children and adolescents including those with gender incongruence, so they should be abridged with gender-affirmative practices. Gender-affirmative care involves pubertal suppression, hormonal therapy, and surgery which should be done in conjugation with the social transition, by a gender-affirmative care team.
Journal of Society of Anesthesiologists of Nepal, Sep 30, 2015
Background: Effective and safe pediatric procedural sedation is still a concern especially in are... more Background: Effective and safe pediatric procedural sedation is still a concern especially in areas outside operation theatres. The aim of the study was to compare the efficacy and safety of oral triclofos and oral midazolam in children undergoing computed tomography. Methods: A prospective randomized double blind study was conducted in 100 children aged one to five years. Group-I (n=50) received oral triclofos 100 mg/kg and Group-II (n=50) oral midazolam 0.75 mg/kg. Both groups were given oral atropine 0.03 mg/kg and supplemented with intravenous midazolam upto 0.1 mg/kg in case of inadequate effect. Onset and duration of sedation, success for completion of procedure and time to recovery were noted. Student's t test and Z test of proportions were used for statistical analysis. Results Majority of children 36(72%) in Group-I achieved Ramsay Sedation Score >4 as compared to 25(50%) in Group-II. Computed tomography scan could be successfully completed at comparable rate (52% vs 56%). Success rate improved to 96% vs 80% after supplementing intravenous midazolam in Group I & II respectively (p< 0.05). Onset (37.91minutes ± 7.96 vs 26 ± 10), duration of sedation (117.91minutes ± 72.41 vs 66.2minutes ± 33) were significantly shorter and recovery (98.19minutes ± 72.58 vs 47.4minutes ± 31.42) in Group I & II respectively was faster in children who received oral midazolam (p< 0.05). Conclusion We conclude that both drugs were equally effective and safe for computed tomography scan in children. However better recovery profile of midazolam makes it more suitable for day care procedures.
Jaypee Brothers Medical Publishers (P) Ltd. eBooks, 2006
PubMed, Nov 1, 2005
The authors have focussed in this article on normal physical growth during puberty and events dur... more The authors have focussed in this article on normal physical growth during puberty and events during adolescence with a brief mention of pubertal disorders.
PubMed, Mar 1, 1998
The development of effective therapy for childhood acute lymphoblastic leukemia (ALL) is one of t... more The development of effective therapy for childhood acute lymphoblastic leukemia (ALL) is one of the undisputed successes in modern oncology. Though it is assumed that children in remission for 6 years after diagnosis are cured(l-3), thereafter relapses do occur, albeit rarely(4-8). We report a case diagnosed to have ALL at
PubMed, Jul 15, 2021
Objective: To assess the efficacy and safety of thalidomide in children with transfusion-dependen... more Objective: To assess the efficacy and safety of thalidomide in children with transfusion-dependent thalassemia. Methods: This prospective, single center, open-label study enrolled children aged 12-18 years, and who received thalidomide for a duration of 6 months at a starting dose of 2-3 mg/kg/day. Efficacy was assessed by reduction in transfusion requirement and rate of fall of hemoglobin. Efficacy was classified as major, moderate and minimal/no response depending on the reduction in transfusion requirement. Safety was assessed by adverse effects related to thalidomide. Results: 37 children [mean (SD) age, 14.7 (1.8) years were included. Rate of fall of hemoglobin reduced from a mean of 1.0 (0.24) g/week pre-thalidomide therapy to 0.58 (0.26) g/week after 6 months of thalidomide (P<0.001). 19 children (51.3%) had major response and 12 (32.4%) had moderate response. In 13.5% and 32.4% children response was observed within the first and second month of therapy, respectively. 15 (40.5%) children remained transfusion - free for a median (IQR) time of 6 (3-10) weeks of thalidomide therapy. Mean serum ferritin (SD) decreased from 1758.9 (835.1) to 1549.6(1016.9) (P<0.001). Mean HbF (SD) showed an increase from 2.95(2.6) to 49.2(33.3) (P<0.001). In 32 children, 47 adverse events were observed. Common adverse events were constipation and neutropenia (mostly mild). Conclusions: Thalidomide resulted in major/moderate response in majority of children with transfusion-dependent thalassemia with satisfactory adverse effect profile.
Journal of Tropical Pediatrics, Apr 1, 2021
Background: Acute kidney injury (AKI) has been recognized as a significant risk factor for mortal... more Background: Acute kidney injury (AKI) has been recognized as a significant risk factor for mortality among adults with severe acute respiratory syndrome coronavirus infection.
Pediatric Blood & Cancer, Aug 13, 2013
Journal of Child Neurology, Apr 1, 2023
Purpose: Nearly 25% to 30% of children with epilepsy develop drug-resistant epilepsy. Etiology of... more Purpose: Nearly 25% to 30% of children with epilepsy develop drug-resistant epilepsy. Etiology of epilepsy, including drug-resistant epilepsy, varies with geographical region. Identifying paucity of etiologic data on drug-resistant epilepsy from our region and similar low-resource settings, we aimed to describe the clinical and etiologic profile of children and adolescents with drug-resistant epilepsy, to better inform region-specific concerns. Methods: A chart-based retrospective review covering 10 years (January 2011–December 2020) was conducted. Participants between 1 months and 18 years of age who fulfilled International League Against Epilepsy (ILAE) definition of drug-resistant epilepsy were enrolled. Clinical details, perinatal history, electroencephalography (EEG), magnetic resonance imaging (MRI), and other evaluation-based data were analyzed. Results: Five hundred ninety-three children (52.3% males) were enrolled. The median age at presentation was 63 (interquartile range [IQR] 12-72) months and median age at onset was 12 (IQR 2-18) months. The most frequent seizure type was generalized (76.6%). Of these, epileptic spasms (48.1%) were most frequent. Focal seizures comprised 22.9%. The predominant contributor to etiology was perinatal adverse events, including perinatal asphyxia (37.9%), neonatal hypoglycemic brain injury (15.6%), and neonatal sepsis/meningitis. Electroclinical syndromes were observed in 361 (60.9%) children. Of these, the most frequent were West syndrome (48%) and Lennox-Gastaut syndrome (6.2%). Conclusion: Perinatal brain injury and brain infections were the most common causes of drug-resistant epilepsy identified. These findings indicate an opportunity for reducing the burden of pediatric drug-resistant epilepsy in our region by instituting preventive measures, including improved perinatal care, promotion of institutional deliveries, optimized obstetric and neonatal care, and immunization for vaccine-preventable infections such as bacterial meningitis and Japanese B encephalitis.
Indian Journal of Youth and Adolescent Health, Jun 26, 2018
Anorexia nervosa (AN) is uncommon in developing societies like India and the clinicians need to h... more Anorexia nervosa (AN) is uncommon in developing societies like India and the clinicians need to have a high index of suspicion to identify it. We are describing an adolescent female who presented with cardiovascular complications (bradycardia and hypotension) of AN. She was initially managed in PICU and later multidisciplinary approach was followed for treating it. This case report reminds us that adolescents having thinness can also have eating disorder.
Journal of Pediatric Hematology Oncology, Apr 1, 2015
Transfusion-dependent thalassemia (TDT) and its treatment affect the physical, emotional, and soc... more Transfusion-dependent thalassemia (TDT) and its treatment affect the physical, emotional, and social functioning, impairing the quality of life (QoL). There are few studies on QoL of adolescent with TDT. Its effect on their siblings' QoL has not been studied so far. In this cross-sectional study, 40 adolescents with TDT, 28 siblings, and 40 controls were studied to assess the QoL of adolescents thalassemics, and their siblings using the shorter version of World Health Organization Quality of Life instrument, the WHOQOL-BREF. Thalassemics had poor perception of their general health and scored significantly lower in all the subscales compared with the controls. Lowest mean subscale scores were for physical (57.7 vs. 72.4, P < 0.001), and psychological domains (56.7 vs. 72.3, P < 0.001). Their siblings also scored significantly less in environment domain. Prevalence of school dropout, short stature, and delayed puberty were significantly higher in thalassemics. Pretransfusion hemoglobin, age at onset of anemia, and chronological age were found to be significant predictors of total summary scores. This study showed that the concept of QoL is a cumulative reflection of individual and disease variables and highlights the negative impact of thalassemia on the patients' QoL and some aspects of their siblings' lives also.
Journal of Pediatric Endocrinology and Metabolism, 2011
Fanconi&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;a... more Fanconi&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s syndrome is a complex of multiple tubular dysfunctions of proximal tubular cells occurring alone or in association with a variety of inherited (primary) or acquired (secondary) disorders. It is characterized by aminoaciduria, normoglycemic glycosuria, tubular proteinuria without hematuria, metabolic acidosis without anion gap and excessive urinary excretion of phosphorous, calcium, uric acid, bicarbonate, sodium, potassium and magnesium. Diabetes insipidus is a disease of collecting tubules and a child mainly presents with dehydration and hypernatremia. We report the first case of idiopathic Fanconi&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s syndrome along with nephrogenic diabetes insipidus (NDI) in a child who presented to us as resistant rickets. Medline search did not reveal any case of nephrogenic diabetes insipidus associated with idiopathic Fanconi&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s syndrome. We hypothesized that the NDI may be due to severe hypokalemia induced tubular dysfunction. The child was treated for hypophosphatemic rickets with severe metabolic acidosis and the treatment for NDI was also given. Now he has healed rickets and normal blood pH, sodium and osmolarity.
Indian Pediatrics, Jun 1, 2021
To study the various comorbidities and their impact on outcome of severe acute respiratory syndro... more To study the various comorbidities and their impact on outcome of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infected children. Methodology: Review of medical records of 120 children (58.4% males), aged 1 month to 18 years, admitted between 1 March and 31 December, 2020 with at least one positive RT-PCR test for SARS-CoV-2. Clinical and demographic variables were compared between children with and without co-morbidities. Results: 62 (51.7%) children had comorbidities. The most common comorbidity was tuberculosis (32.3%) followed by other infections (27.4%) and hematological (19.4%) conditions. Fever (89.2%) was the most common clinical feature followed by respiratory (52.5%) and gastrointestinal (32.5%) manifestations. There was no significant difference in the severity of COVID illness, length of hospital stay and adverse outcomes (ventilation and mortality) among children with and without comorbidities. Conclusion: The presence of a comorbid illness in pediatric inpatients with COVID-19 did not impact the illness severity, length of hospitalization, ventilation requirement and mortality.
Research Square (Research Square), May 12, 2023
Background Bioavailable vitamin D levels is could be a better marker than total 25 hydroxy vitami... more Background Bioavailable vitamin D levels is could be a better marker than total 25 hydroxy vitamin D levels to assess vitamin D status in children with nephrotic syndrome. Primary objective To assess the levels of Serum bioavailable vitamin D in children aged 1 to 12 years with idiopathic FENS and in healthy controls. Secondary objective 1. To measure the levels of bioavailable vitamin D in FENS and after 4 weeks of standard steroid therapy induced remission. 2. To compare levels of serum and urine VDBP in FENS and after 4 weeks of standard steroid therapy induced remission. Materials and Methods A longitudinal study was conducted in children between age 1 to 12 years with idiopathic rst episode nephrotic syndrome. After diagnosis of nephrotic syndrome as per ISPN guidelines, additional investigations like calcium, phosphorus, ALP, 25 hydroxy vitamin D, PTH, serum Vitamin D binding protein (VDBP) and urinary VDBP. Bioavailable and free vitamin D was calculated using above data. The patients were followed up after 4 weeks of remission and investigations were repeated and bioavailable vitamin D was calculated again, later results were compared and analysed. Appropriate statistical tests were applied for parametric and non-parametric data. P value of less than 0.05 was considered statistically signi cant. Results: The mean 25 hydroxy vitamin D level was 11.27(6.08) at FENS and at 4 weeks follow-up it was 13.65 (6.24). Hence children with FENS remained de cient in 25 hydroxy vitamin D, both during relapse and remission compared to healthy controls (15.97 ng/ml). The mean serum VDBP level in FENS during relapse was 242.90 (127.75). There was signi cant correlation of serum VDBP with serum albumin levels (p value 0.04). At 4 weeks of remission the VDBP levels increased to 550.7(219.7), this increase was signi cant (p value <0.001). The mean free vitamin D at FENS was 1.54 (1.70) pg/ml, on follow-up visit at 4 weeks of remission the free vitamin D levels decreased to 0.68 (0.53) pg/ml. The mean bioavailable vitamin D in FENS during relapse was 0.75 (0.84) ng/ml and on follow-up at 4 weeks of remission increased to 1.11 (0.84) ng/ml (p value = 0.015). Conclusion Page 3/12 Children with FENS are de cient of vitamin D levels. The free and bioavailable vitamin D levels are reduced in children with FENS during proteinuria, as compared to healthy controls. Further studies showing correlation of bioavailable vitamin D and 25 hydroxyvitamin D with bone mineral density are required in children to validate the usage of bioavailable vitamin D in clinical practice.
PubMed, May 14, 2023
Justification: The transgender community has been long stigmatized, and discriminated against, an... more Justification: The transgender community has been long stigmatized, and discriminated against, and faces numerous mental and physical problems. Certain indicators of transgender personality appear during childhood and more often before puberty begins. This puts the onus on Pediatricians to identify and offer evidence-based care for their benefit. There is an urgent and deep-felt need to understand the medical, legal, and social aspects of the care of transgender children. Hence, Adolescent Health Academy decided to release a statement on the care of transgender children, adolescents, and youth. Objectives: To review the existing international and national guidelines and recommendations to formulate a statement for the Pediatricians on (a) terminologies and definitions; (b) legal status in India; and (c) implications for pediatric practice. Process: A task force was convened by the Adolescent Health Academy as the writing committee to draft the guidelines. These were approved by all the members of the task force and the Executive Board of Adolescent Health Academy (2022). Recommendations: Gender identity develops in childhood and adolescence as a feeling of self, and it should be respected to mitigate gender dysphoria. The law permits transgenders the right of self-affirmation and it upholds their dignity in society. The transgender community is prone to victimization, and prejudice leading to a high risk of substance abuse, suicidal ideation, and mental health issues. Pediatricians are the primary care providers of children and adolescents including those with gender incongruence, so they should be abridged with gender-affirmative practices. Gender-affirmative care involves pubertal suppression, hormonal therapy, and surgery which should be done in conjugation with the social transition, by a gender-affirmative care team.
Journal of Society of Anesthesiologists of Nepal, Sep 30, 2015
Background: Effective and safe pediatric procedural sedation is still a concern especially in are... more Background: Effective and safe pediatric procedural sedation is still a concern especially in areas outside operation theatres. The aim of the study was to compare the efficacy and safety of oral triclofos and oral midazolam in children undergoing computed tomography. Methods: A prospective randomized double blind study was conducted in 100 children aged one to five years. Group-I (n=50) received oral triclofos 100 mg/kg and Group-II (n=50) oral midazolam 0.75 mg/kg. Both groups were given oral atropine 0.03 mg/kg and supplemented with intravenous midazolam upto 0.1 mg/kg in case of inadequate effect. Onset and duration of sedation, success for completion of procedure and time to recovery were noted. Student's t test and Z test of proportions were used for statistical analysis. Results Majority of children 36(72%) in Group-I achieved Ramsay Sedation Score >4 as compared to 25(50%) in Group-II. Computed tomography scan could be successfully completed at comparable rate (52% vs 56%). Success rate improved to 96% vs 80% after supplementing intravenous midazolam in Group I & II respectively (p< 0.05). Onset (37.91minutes ± 7.96 vs 26 ± 10), duration of sedation (117.91minutes ± 72.41 vs 66.2minutes ± 33) were significantly shorter and recovery (98.19minutes ± 72.58 vs 47.4minutes ± 31.42) in Group I & II respectively was faster in children who received oral midazolam (p< 0.05). Conclusion We conclude that both drugs were equally effective and safe for computed tomography scan in children. However better recovery profile of midazolam makes it more suitable for day care procedures.
Jaypee Brothers Medical Publishers (P) Ltd. eBooks, 2006
PubMed, Nov 1, 2005
The authors have focussed in this article on normal physical growth during puberty and events dur... more The authors have focussed in this article on normal physical growth during puberty and events during adolescence with a brief mention of pubertal disorders.
PubMed, Mar 1, 1998
The development of effective therapy for childhood acute lymphoblastic leukemia (ALL) is one of t... more The development of effective therapy for childhood acute lymphoblastic leukemia (ALL) is one of the undisputed successes in modern oncology. Though it is assumed that children in remission for 6 years after diagnosis are cured(l-3), thereafter relapses do occur, albeit rarely(4-8). We report a case diagnosed to have ALL at
PubMed, Jul 15, 2021
Objective: To assess the efficacy and safety of thalidomide in children with transfusion-dependen... more Objective: To assess the efficacy and safety of thalidomide in children with transfusion-dependent thalassemia. Methods: This prospective, single center, open-label study enrolled children aged 12-18 years, and who received thalidomide for a duration of 6 months at a starting dose of 2-3 mg/kg/day. Efficacy was assessed by reduction in transfusion requirement and rate of fall of hemoglobin. Efficacy was classified as major, moderate and minimal/no response depending on the reduction in transfusion requirement. Safety was assessed by adverse effects related to thalidomide. Results: 37 children [mean (SD) age, 14.7 (1.8) years were included. Rate of fall of hemoglobin reduced from a mean of 1.0 (0.24) g/week pre-thalidomide therapy to 0.58 (0.26) g/week after 6 months of thalidomide (P<0.001). 19 children (51.3%) had major response and 12 (32.4%) had moderate response. In 13.5% and 32.4% children response was observed within the first and second month of therapy, respectively. 15 (40.5%) children remained transfusion - free for a median (IQR) time of 6 (3-10) weeks of thalidomide therapy. Mean serum ferritin (SD) decreased from 1758.9 (835.1) to 1549.6(1016.9) (P<0.001). Mean HbF (SD) showed an increase from 2.95(2.6) to 49.2(33.3) (P<0.001). In 32 children, 47 adverse events were observed. Common adverse events were constipation and neutropenia (mostly mild). Conclusions: Thalidomide resulted in major/moderate response in majority of children with transfusion-dependent thalassemia with satisfactory adverse effect profile.
Journal of Tropical Pediatrics, Apr 1, 2021
Background: Acute kidney injury (AKI) has been recognized as a significant risk factor for mortal... more Background: Acute kidney injury (AKI) has been recognized as a significant risk factor for mortality among adults with severe acute respiratory syndrome coronavirus infection.
Pediatric Blood & Cancer, Aug 13, 2013
Journal of Child Neurology, Apr 1, 2023
Purpose: Nearly 25% to 30% of children with epilepsy develop drug-resistant epilepsy. Etiology of... more Purpose: Nearly 25% to 30% of children with epilepsy develop drug-resistant epilepsy. Etiology of epilepsy, including drug-resistant epilepsy, varies with geographical region. Identifying paucity of etiologic data on drug-resistant epilepsy from our region and similar low-resource settings, we aimed to describe the clinical and etiologic profile of children and adolescents with drug-resistant epilepsy, to better inform region-specific concerns. Methods: A chart-based retrospective review covering 10 years (January 2011–December 2020) was conducted. Participants between 1 months and 18 years of age who fulfilled International League Against Epilepsy (ILAE) definition of drug-resistant epilepsy were enrolled. Clinical details, perinatal history, electroencephalography (EEG), magnetic resonance imaging (MRI), and other evaluation-based data were analyzed. Results: Five hundred ninety-three children (52.3% males) were enrolled. The median age at presentation was 63 (interquartile range [IQR] 12-72) months and median age at onset was 12 (IQR 2-18) months. The most frequent seizure type was generalized (76.6%). Of these, epileptic spasms (48.1%) were most frequent. Focal seizures comprised 22.9%. The predominant contributor to etiology was perinatal adverse events, including perinatal asphyxia (37.9%), neonatal hypoglycemic brain injury (15.6%), and neonatal sepsis/meningitis. Electroclinical syndromes were observed in 361 (60.9%) children. Of these, the most frequent were West syndrome (48%) and Lennox-Gastaut syndrome (6.2%). Conclusion: Perinatal brain injury and brain infections were the most common causes of drug-resistant epilepsy identified. These findings indicate an opportunity for reducing the burden of pediatric drug-resistant epilepsy in our region by instituting preventive measures, including improved perinatal care, promotion of institutional deliveries, optimized obstetric and neonatal care, and immunization for vaccine-preventable infections such as bacterial meningitis and Japanese B encephalitis.
Indian Journal of Youth and Adolescent Health, Jun 26, 2018
Anorexia nervosa (AN) is uncommon in developing societies like India and the clinicians need to h... more Anorexia nervosa (AN) is uncommon in developing societies like India and the clinicians need to have a high index of suspicion to identify it. We are describing an adolescent female who presented with cardiovascular complications (bradycardia and hypotension) of AN. She was initially managed in PICU and later multidisciplinary approach was followed for treating it. This case report reminds us that adolescents having thinness can also have eating disorder.
Journal of Pediatric Hematology Oncology, Apr 1, 2015
Transfusion-dependent thalassemia (TDT) and its treatment affect the physical, emotional, and soc... more Transfusion-dependent thalassemia (TDT) and its treatment affect the physical, emotional, and social functioning, impairing the quality of life (QoL). There are few studies on QoL of adolescent with TDT. Its effect on their siblings' QoL has not been studied so far. In this cross-sectional study, 40 adolescents with TDT, 28 siblings, and 40 controls were studied to assess the QoL of adolescents thalassemics, and their siblings using the shorter version of World Health Organization Quality of Life instrument, the WHOQOL-BREF. Thalassemics had poor perception of their general health and scored significantly lower in all the subscales compared with the controls. Lowest mean subscale scores were for physical (57.7 vs. 72.4, P < 0.001), and psychological domains (56.7 vs. 72.3, P < 0.001). Their siblings also scored significantly less in environment domain. Prevalence of school dropout, short stature, and delayed puberty were significantly higher in thalassemics. Pretransfusion hemoglobin, age at onset of anemia, and chronological age were found to be significant predictors of total summary scores. This study showed that the concept of QoL is a cumulative reflection of individual and disease variables and highlights the negative impact of thalassemia on the patients' QoL and some aspects of their siblings' lives also.
Journal of Pediatric Endocrinology and Metabolism, 2011
Fanconi&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;a... more Fanconi&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s syndrome is a complex of multiple tubular dysfunctions of proximal tubular cells occurring alone or in association with a variety of inherited (primary) or acquired (secondary) disorders. It is characterized by aminoaciduria, normoglycemic glycosuria, tubular proteinuria without hematuria, metabolic acidosis without anion gap and excessive urinary excretion of phosphorous, calcium, uric acid, bicarbonate, sodium, potassium and magnesium. Diabetes insipidus is a disease of collecting tubules and a child mainly presents with dehydration and hypernatremia. We report the first case of idiopathic Fanconi&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s syndrome along with nephrogenic diabetes insipidus (NDI) in a child who presented to us as resistant rickets. Medline search did not reveal any case of nephrogenic diabetes insipidus associated with idiopathic Fanconi&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39;s syndrome. We hypothesized that the NDI may be due to severe hypokalemia induced tubular dysfunction. The child was treated for hypophosphatemic rickets with severe metabolic acidosis and the treatment for NDI was also given. Now he has healed rickets and normal blood pH, sodium and osmolarity.
Indian Pediatrics, Jun 1, 2021
To study the various comorbidities and their impact on outcome of severe acute respiratory syndro... more To study the various comorbidities and their impact on outcome of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infected children. Methodology: Review of medical records of 120 children (58.4% males), aged 1 month to 18 years, admitted between 1 March and 31 December, 2020 with at least one positive RT-PCR test for SARS-CoV-2. Clinical and demographic variables were compared between children with and without co-morbidities. Results: 62 (51.7%) children had comorbidities. The most common comorbidity was tuberculosis (32.3%) followed by other infections (27.4%) and hematological (19.4%) conditions. Fever (89.2%) was the most common clinical feature followed by respiratory (52.5%) and gastrointestinal (32.5%) manifestations. There was no significant difference in the severity of COVID illness, length of hospital stay and adverse outcomes (ventilation and mortality) among children with and without comorbidities. Conclusion: The presence of a comorbid illness in pediatric inpatients with COVID-19 did not impact the illness severity, length of hospitalization, ventilation requirement and mortality.
Research Square (Research Square), May 12, 2023
Background Bioavailable vitamin D levels is could be a better marker than total 25 hydroxy vitami... more Background Bioavailable vitamin D levels is could be a better marker than total 25 hydroxy vitamin D levels to assess vitamin D status in children with nephrotic syndrome. Primary objective To assess the levels of Serum bioavailable vitamin D in children aged 1 to 12 years with idiopathic FENS and in healthy controls. Secondary objective 1. To measure the levels of bioavailable vitamin D in FENS and after 4 weeks of standard steroid therapy induced remission. 2. To compare levels of serum and urine VDBP in FENS and after 4 weeks of standard steroid therapy induced remission. Materials and Methods A longitudinal study was conducted in children between age 1 to 12 years with idiopathic rst episode nephrotic syndrome. After diagnosis of nephrotic syndrome as per ISPN guidelines, additional investigations like calcium, phosphorus, ALP, 25 hydroxy vitamin D, PTH, serum Vitamin D binding protein (VDBP) and urinary VDBP. Bioavailable and free vitamin D was calculated using above data. The patients were followed up after 4 weeks of remission and investigations were repeated and bioavailable vitamin D was calculated again, later results were compared and analysed. Appropriate statistical tests were applied for parametric and non-parametric data. P value of less than 0.05 was considered statistically signi cant. Results: The mean 25 hydroxy vitamin D level was 11.27(6.08) at FENS and at 4 weeks follow-up it was 13.65 (6.24). Hence children with FENS remained de cient in 25 hydroxy vitamin D, both during relapse and remission compared to healthy controls (15.97 ng/ml). The mean serum VDBP level in FENS during relapse was 242.90 (127.75). There was signi cant correlation of serum VDBP with serum albumin levels (p value 0.04). At 4 weeks of remission the VDBP levels increased to 550.7(219.7), this increase was signi cant (p value <0.001). The mean free vitamin D at FENS was 1.54 (1.70) pg/ml, on follow-up visit at 4 weeks of remission the free vitamin D levels decreased to 0.68 (0.53) pg/ml. The mean bioavailable vitamin D in FENS during relapse was 0.75 (0.84) ng/ml and on follow-up at 4 weeks of remission increased to 1.11 (0.84) ng/ml (p value = 0.015). Conclusion Page 3/12 Children with FENS are de cient of vitamin D levels. The free and bioavailable vitamin D levels are reduced in children with FENS during proteinuria, as compared to healthy controls. Further studies showing correlation of bioavailable vitamin D and 25 hydroxyvitamin D with bone mineral density are required in children to validate the usage of bioavailable vitamin D in clinical practice.