Critical Illness Polyneuropathy in a Child: A Case Report.

Critical illness polyneuropathy (CIP) and myopathy (CIM) are underreported conditions in critically ill children with prolonged intensive care unit stays and mechanical ventilation. We report a case of a 10-year-old boy with pneumococcal meningoencephalitis with severe sepsis and multiorgan dysfunction. The child required prolonged ventilation, sedation, and inotropic support. He had repeated extubation failures and the development of quadriparesis with areflexia. Electrophysiology studies were consistent with CIP with acute motor and sensory axonal polyneuropathy and elevated muscle enzymes. He was treated with supportive measures and physiotherapy along with management of the underlying condition. He recovered slowly over 68 days with a good recovery with a modified Rankin's scale score of 4 on discharge. There is a need to pay attention to all critically ill children and should have a high index of suspicion for the development of CIP/CIM which can have an impact on course and outcome.

Early-Onset Takayasu Arteritis in Childhood: A Case Report.

Takayasu arteritis is a chronic, idiopathic, inflammatory disease mainly affecting medium and large vessels with a significant rate of morbidity and mortality. The vessels most frequently affected are the aorta and its branches; branches originating from the aortic arch include right brachiocephalic trunk and its branches, left common carotid artery, left subclavian artery, coronary arteries from the ascending aorta, celiac trunk, right and left renal arteries, superior and inferior mesenteric arteries from the descending aorta, and right and left iliofemoral arteries. Local and systemic inflammation along with end organ ischemia is attributed to severe clinical manifestations associated with this condition. Although Takayasu arteritis is more commonly diagnosed in adults, this study highlights the unusual occurrence of childhood-onset Takayasu arteritis (TAK), presenting a unique set of diagnostic challenges. We present a case of a seven-year-old female patient who manifested atypical symptoms, such as absent pulses and malignant hypertension at an early age, leading to a delayed diagnosis. The patient's clinical course, including diagnostic workup and imaging studies such as CT or MR angiography, is thoroughly discussed. This study emphasizes the importance of recognizing the subtleties of Takayasu arteritis in children. The disease may initially masquerade as other common conditions, such as peripheral arterial disease, coarctation of aorta, renal artery stenosis, chronic renal disease, and increased intracranial pressure, thereby hindering timely diagnosis and appropriate intervention. This case underscores the importance of considering Takayasu arteritis as a differential diagnosis in children, presenting with unexplained constitutional symptoms or signs of systemic vasculitis, emphasizing the need for multidisciplinary collaboration and tailored therapeutic intervention to optimize the outcome in this rare and potentially debilitating condition.

Late-Onset Dilated Cardiomyopathy in Auto Immune-Mediated Complete Congenital Heart Block: A Case Report.

Complete congenital heart block (CHB), a rare and fatal bradyarrhythmia observed in children, carries significant mortality and morbidity. When congenital heart block occurs in isolation with a structurally normal heart, it prompts suspicion of an autoimmune etiology, wherein maternal antibodies are transmitted transplacentally, impacting the fetal conducting system. The manifestation of congenital complete atrioventricular block (CCAVB) can lead to complications such as dilated cardiomyopathies, arrhythmias, and fibroelastosis in certain cases. Notably, dilated cardiomyopathy is a significant prognostic factor in children diagnosed with congenital heart block. Pathological investigations have revealed the presence of antibodies, complements, and indicators of inflammation or fibrosis across the myocardium, emphasizing the shared molecular mechanisms between CCAVB and the development of dilated cardiomyopathy (DCM). This article presents the case of a one-year-old female child who presented with signs of dilated cardiomyopathy, later identified through retrospective evaluation as having autoimmune congenital heart block. The mother of the child was diagnosed with Sjogren's syndrome, characterized by positive anti-RO titers. Remarkably, the child remained asymptomatic for a year without the need for pacing intervention. The child's condition was successfully stabilized with appropriate treatment, and plans for pacemaker insertion will be considered once specific criteria are met. The onset of cardiomyopathy in a known case of CCAVB should serve as a crucial alert for prognostic considerations and the potential necessity for early-pacing intervention.

A Rare Case of Isolated Congenital Facial Nerve Aplasia in an Infant.

Facial nerve aplasia is an exceptionally rare condition, with only a few reported cases in the existing medical literature. Congenital facial palsy (CFP) is characterized by the clinical manifestation of facial paralysis involving the seventh cranial nerve, either evident at birth or shortly thereafter. This condition is categorized based on various parameters, including the presence of trauma or developmental origins, unilateral or bilateral involvement, and whether the paralysis is complete or incomplete. While CFP is uncommon, its occurrence can pose multiple challenges for newborns, such as difficulties in nursing and incomplete closure of the affected eye. In cases where the paralysis persists, there is the potential for a long-term impact on the child's speech, emotional expressions, and mastication. Here we present the case of a six-month-old male child who experienced lower motor neuron palsy of the facial nerve on the left side since birth. This case contributes to the limited knowledge surrounding facial nerve aplasia and CFP, emphasizing the importance of early diagnosis and intervention to mitigate potential long-term complications.

A Comprehensive Echocardiographic Assessment of Neonatal Right Ventricular Function in Neonatal Intensive Care Unit Babies.

Background The right ventricle (RV) in the fetus is the predominant chamber, accounting for about 60% of the total cardiac output. The majority of the RV outflow volume is diverted from the pulmonary artery via the ductus arteriosus to the descending aorta. After birth, the RV undergoes extensive structural and functional modifications. The RV undergoes an improper transition from fetal to neonatal circulation in sick neonatal intensive care unit (NICU) babies. Functional echocardiography is now commonly being used in most NICUs as it is a noninvasive and bedside investigation that gives an immediate evaluation of hemodynamics and can be taken into consideration as an extension of clinical assessment to study a critically unwell neonate. Therefore, a study of RV functions in NICU neonates will help in better understanding the neonatal cardiopulmonary response to different diseases. Thus, this study aimed to assess RV functions in neonates getting admitted to the NICU of a tertiary care institute. Methodology This observational, cross-sectional study was approved by the Research & Recognition Committee of Dr. D. Y. Patil Vidyapeeth, Pune. In total, 35 cases of term neonates admitted to the NICU at Dr. D. Y. Patil Medical College, Hospital & Research Centre, Pune who fulfilled the inclusion criteria were enrolled in this study after obtaining consent from their parents. Two-dimensional echocardiography was performed by a trained pediatric cardiologist, and the findings were substantiated by a neonatologist trained in echocardiography. Results Our study found a strong association between tricuspid inflow velocity and neonates with sepsis. Similarly, a significant association was observed between abnormal tricuspid Inflow velocity (E/A and E/E') and neonates requiring inotropic support. Conclusions Data on the normal values of different echocardiographic parameters of the systolic and diastolic function of the RV during the neonatal phase of life are currently limited. Our data offer preliminary insights into this topic. Early echocardiography and intervention are advisable, especially in neonates with sepsis and requiring inotropic support.

Effect of nutritional counselling for using anthropometric indices among Indians.

Malnutrition in children under 5 years is a major public health problem in developing countries. Malnutrition complex comprises of under-nutrition including multiple conditions like acute, chronic malnutrition, micronutrient deficiencies and nutrition related to obesity. Therefore, it is of interest to report data on the individualized nutritional counselling on nutritional status among mild to moderately malnourished children aged 2 - 5 years at one, three and six month follow up. Their dietary habits will help to determine the aetiology of mild/moderate malnutrition.150 Children attending outpatient department of the Department of Paediatrics of D Y Patil Medical College and admitted with mild/moderate malnutrition were included in the study after taking informed consent from their parents. The children were randomly allocated into 4 groups (group 1 - 4). The groups consisted of children where dedicated nutritional counselling was provided at 1 month, 1 and 3 month follow up and where-in no dedicated counselling was provided. A diet chart was provided with counselling. Group 4 followed up with routine care without any dietary intervention. Detailed dietary, socio economic history, clinical examination with anthropometry was done followed-up at 1, 3, 6 months from date of inclusion. Majority (57.3%) belonged to lower socio-economic class according to Kuppuswamy scale. The mean birth weight was 2.4 kg, age 34.5 months and age of weaning 7.6 months. Data shows that 70% children had mild malnutrition and 30% moderate malnutrition. At 6 month follow up amongst 105 children with mild malnutrition, 82 still had mild malnutrition, 4 normal, 19 had moderate malnutrition. Under-five childhood malnutrition is highly prevalent in poor socioeconomic strata of the society. Nutritional counselling provided by trained healthcare providers in existing settings are effective in improving nutritional status, daily calorie/protein intake, prevention of malnutrition. Prevention/treatment of co-existing illness bears equal importance.

Quadratic and Cubic Optical Nonlinearities of Y-Shaped and Distorted-H-Shaped Arylalkynylruthenium Complexes.

Straightforward syntheses of bis[bis{1,2-bis(diphenylphosphino)ethane}ruthenium]-functionalized 1,3,5-triethynylbenzene-cored complexes via a methodology employing "steric control" permit facile formation of Y-shaped Sonogashira coupling products and distorted-H-shaped homo-coupled quadrupolar products. Cyclic voltammetric data from these products reveal two reversible metal alkynyl-localized oxidation processes for all complexes. The wavelengths of the linear optical absorption maxima are dominated by the nature of the peripheral alkynyl ligand rather than the substituent at the unique arm of the "Y" or at the quadrupolar complex "core". The quadratic optical nonlinearities of the Y-shaped complexes were assessed by the hyper-Rayleigh scattering technique at 800 nm and employing 100 fs light pulses; introduction of donor NEt2 and/or acceptor NO2 to the wedge periphery resulted in non-zero nonlinearities, with the largest ßHRS,800 values being observed for the complexes containing the 4-nitrophenylalkynyl ligands. Depolarization ratios are consistent with substantial off-diagonal first hyperpolarizability tensor components and 2D nonlinear character. Computational studies employing time-dependent density functional theory have been employed to assign the key low-energy transitions in the linear optical spectra and to compute the quadratic nonlinear optical tensorial components. Cubic optical nonlinearities of the quadrupolar complexes were assessed by the Z-scan technique over the range 500-1600 nm and employing 130 fs light pulses; two-photon absorption cross-sections for these distorted-H-shaped complexes are moderate to large in value (up to 5500 GM at 880 nm), while one example displays significant three-photon absorption (1300×10-80  cm6 s2 at 1200 nm).

α-Geminal dihydroxymethyl piperidine and pyrrolidine iminosugars: synthesis, conformational analysis, glycosidase inhibitory activity, and molecular docking studies.

The Jocic-Reeve and Corey-Link type reaction of dichloromethyllithium with suitably protected 5-keto-hexofuranoses followed by treatment with sodium azide and sodium borohydride reduction gave 5-azido-5-hydroxylmethyl substituted hexofuranoses 7a-c with required geminal dihydroxymethyl group. Removal of protecting groups and converting the C-1 anomeric carbon into free hemiacetal followed by intramolecular reductive aminocyclization with in situ generated C5-amino functionality afforded corresponding 5C-dihydroxymethyl piperidine iminosugars 2a-c. Alternatively, removal of protecting groups in 7b and 7c and chopping of C1-anomeric carbon gave C2-aldehyde that on intramolecular reductive aminocyclization with C5-amino gave 4C-dihydroxymethyl pyrrolidine iminosugars 1b and 1c, respectively. On the basis of the (1)H NMR studies, the conformations of 2a/2b were assigned as (4)C(1) and that of 2c as (1)C(4). The glycosidase inhibitory activities of all five iminosugars were studied with various glycosidase enzymes and compared with natural d-gluco-1-deoxynojirimycin (DNJ). All the five compounds were found to be potent inhibitors of rice α-glucosidase with K(i) and IC(50) values in the nanomolar concentration range. Iminosugars 2b and 1b were found to be more potent inhibitors than their parent iminosugar. These results were substantiated by in silico molecular docking studies.

Bicyclic amino acid-carbohydrate-conjugates as conformationally restricted hydroxyethylamine (HEA) transition-state isosteres.

This communication describes a general synthetic route to bicyclic amino acid-carbohydrate-conjugates, which would be useful as conformationally restricted hydroxyethylamine (HEA) transition-state isosteres. The synthesis was achieved in 12 steps starting from D-glucose. The striking features of this system are the bicyclic rigid core displaying an α-amino acid side chain and hydroxyethylamine moiety--both of which would be potentially important for receptor interactions, leading to various biomedical responses, as described in the literature. Crystal structure investigation suggested extensive intermolecular hydrogen-bonding interactions in this system, involving the backbone amide and hydroxyl groups.

Synthesis of new six- and seven-membered 1-N-iminosugars as promising glycosidase inhibitors.

New six- and seven-membered 1-N-iminosugars were prepared from d-glucose by the stereoselective Michael addition of nitromethane to d-glucose derived α,ß-unsaturated ester A followed by one pot reduction of nitro/ester functionality and subsequent amine protection to get N-Cbz protected aminol 6. Hydrolysis of 1,2-acetonide and reductive aminocyclization gave seven membered 1-N-iminosugar 5b. While, hydrolysis of 1,2-acetonide followed by NaIO(4) oxidative cleavage and hydrogenation using 10% Pd(OH)(2)/C, H(2) gave six membered 1-N-iminosugar 4a; the hydrogenation using 10% Pd/C-H(2) however, gave N-methyl substituted 1-N-iminosugar 4b. The hydrochloride salts of 4a/4b and 5b were found to be specific α-galactosidase and moderate α-glucosidae inhibitors, respectively, in micro molar range.

Intramolecular reductive cyclization strategy to the synthesis of (-)-6-methyl-3-hydroxy-piperidine-2-carboxylic acid, (+)-6-methyl-(2-hydroxymethyl)-piperidine-3-ol and their glycosidase inhibitory activity.

The first stereoselective synthesis of (2S,3R,6S)-6-methyl-3-hydroxy-piperidine-2-carboxylic acid (-)-6 and (2R,3R,6S)-6-methyl-(2-hydroxymethyl)-piperidine-3-ol (+)-7 was achieved starting from readily available d-glucose in 14 steps with 17% overall yield for both the compounds. The key feature of the present strategy includes the Wittig-olefination for the preparation of required conjugated keto-azide 9 and construction of 2,3,6-trisubstituted piperidine skeleton 11 by applying intramolecular reductive cyclization of conjugated keto-azide intermediate. The glycosidase inhibitory activity of compounds 6 and 7 towards several glycosidases has been evaluated.

A versatile access to calystegine analogues as potential glycosidases inhibitors.

An efficient metathetic strategy and nitrone chemistry have been suitably tethered to construct 8-azabicyclo[3.2.1]octanes as versatile precursors for the synthesis of several calystegine analogues. This synthetic strategy relies on the ability of mannose-derived nitrone to undergo a highly stereoselective nucleophilic addition of various Grignard reagents to access syn orientation of alkenes, which then smoothly undergo ring-closing metathesis (RCM) to provide this framework. These RCM products 18 and 20 have been successfully used as advance precursors to synthesize many calystegine analogues (27, 36, 38, 40, 43, and 44) either by syn-dihydroxylation or by hydrogenation and followed by global deprotection. Interestingly, both compounds 36 and 40 exhibited significant noncompetitive inhibition against alpha-mannosidase and N-acetyl-beta-D-glucosaminidase.

Hepatitis B vaccination in premature and low birth weight (LBW) babies.

OBJECTIVE: To assess the immune response of preterm and low birth weight babies (LBW) to hepatitis B (HB) vaccine. SETTING: Neonatal Intensive Care Unit (NICU), postnatal ward and follow up clinics of KEM Hospital, Pune. DESIGN: Open trial. METHODS: 100 babies were enrolled in four study groups. Group I - preterm, gestational age (GA) < 34 weeks; Group II - GA 34 to 36 weeks; Group III full term <2.5 kg (LBW babies); and Group IV full term >2.5 kg (controls). A recombinant DNA HB vaccine was given at 0, 1, 2 and 12 month schedule. The first injection was administered as soon as the neonate was stabilized. Immune response in terms of anti HBs titres (AUSAB EIA Diagnostic kit) was measured one month after each of the first three injections and at the time of one year booster. Adverse events were monitored. RESULTS: 88 and 62 babies completed the study till the third dose and one year booster dose respectively. Immune response of HB vaccine was uniformly good in all the study groups with 100 % sero-conversion after the second dose itself. By one year (i.e. before the booster dose), very high titres were recorded in all 100%, with 85% demonstrating titres >1000 mIU/ml. Preterm and LBW babies had higher GMT as compared to full term babies till one month after third dose. By one year (before booster), full term babies had higher GMT than preterm and LBW babies. However, these differences were not statistically significant. The vaccine was well tolerated and safe and there were no adverse reactions. CONCLUSION: Immune response of preterm, LBW and full term babies to the new generation recombinant DNA HB vaccine was uniformly good. High and long term seroprotective levels were achieved after the second dose itself.