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We describe 2 expremature infants presenting with SARS-CoV-2-related pulmonary disease in their second and fifth week of life needing support with mechanical ventilation. Both infants' initial presentation was with repeated apneas. These cases highlight that SARS-CoV-2 infection could present with apneas and has the potential to progress to more severe pulmonary disease in this high-risk age group of patients. Both patients were treated with remdesivir (RDV). We provide the data of 2 high-risk neonates successfully treated with RDV without observation of any described side effects. A recognition that these high-risk neonates could deteriorate and early multidisciplinary team discussion is the mainstay to the compassionate access to RDV. Our experience led us to develop a guideline on the use of RDV below 12 years of age, with particular focus on infants and young children.

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INTRODUCTION: Haemolytic uremic syndrome (HUS) is one of the most common causes of acute renal failure in children but it is uncommon in newborns. To our knowledge only five cases have been reported so far (probably underreported). The known modalities of treatment include transfusion of plasma and plasmapheresis. We report a case of neonatal HUS for whom we performed an exchange transfusion to good effect. CASE DESCRIPTION: A term vaginally born baby, meconium stained and floppy at birth presented with severe anaemia in the first few hours of life. The baby later on developed renal failure and blood picture was suggestive of severe thrombocytopenia and microangiopathic haemolytic anaemia. No extra renal manifestations of birth asphyxia were noted. A double volume exchange transfusion was performed relatively early and subsequently platelet and haemoglobin stabilised and renal failure improved. DISCUSSION AND EVALUATION: The clinical impression in this case was convincing of neonatal HUS, likely attributable to birth asphyxia but needs to be differentiated from disseminated intravascular coagulation (DIC) and thrombotic thrombocytopenic purpura (TTP). The coagulation profile is usually normal in HUS but it is abnormal in DIC, whereas in TTP one would find hyperbilirubinemia, increased creatinine, haemolysis etc. TTP is rare but not very uncommon in infancy. Congenital TTP is attributed to an inherent deficiency of ADAMTS-13, which is a vWF-cleaving metalloprotease. Irrespective of the etiology of HUS in our case, a dramatic response was observed with exchange transfusion. Transfusion of fresh frozen plasma (FFP) and plasmapheresis are known treatment modalities. FFP replaces the missing or altered complement factors and plasmapheresis removes antibodies, immune complexes and toxins. An exchange transfusion combines both these functions. CONCLUSIONS: In the absence of facilities for plasmapheresis, exchange transfusion is a good alternative.

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BACKGROUND: Extubation failure (EF), defined as need for re-intubation within 24-72 h, is multifactorial. Factors predicting EF in adults generally are not useful in children. OBJECTIVE: To determine the factors associated with EF and to facilitate prediction of EF in mechanically ventilated infants and children less than 12 years of age. MATERIAL AND  METHODS: Design Prospective cohort study. Setting PICU and NICU of a multispecialty tertiary care institute. Patients All consecutive newborns, infants and children, who remained on the ventilator for more than 12 h, were included. Patients with upper airway obstruction, neuromuscular disorders, complex anatomic malformations, accidental extubation, tracheostomy or death before extubation were excluded. Methods The pre-extubation clinical, laboratory and ventilatory parameters were collected for 92 cases over a one and half year period. The EF rate was calculated for each variable using STATA 9. All the treating physicians were blinded to the data collection procedure. MEASUREMENTS AND RESULTS: Demographics were comparable between the extubation success and EF groups. Respiratory failure was the main cause requiring ventilation (46.74 %, 95 % CI 0.37-0.57) as well as EF (30.23 %, 95 % CI 0.08-0.23). 76.92 % (95 % CI 0.58-0.89) of patients that failed extubation had alterations in respiratory effort, 38.46 % (95 % CI 0.22-0.57) each had either poor or increased respiratory effort. Poor cough reflex (p = 0.001), thick endotracheal secretions (p = 0.02), failed spontaneous breathing trial (SBT) (p = 0.001) and higher rapid shallow breathing index (RSBI) (p = 0.001) were found to be associated with EF. CONCLUSIONS: Paediatric EF is multifactorial. Increased or poor respiratory effort and failed SBT are potential factors in deciding re-intubation. Increased RSBI, poor cough reflex and thick.

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Juvenile dermatomyositis (JDM) is a rare childhood disease with autoimmune association. Environmental factors are known to trigger JDM in genetically susceptible individuals (Schmieder et al., Dermatol Online 6:3, 2009). Calcinosis is a well-established complication of JDM. Prevalence is higher in children (30-70%; Özkaya et al., Erciyes Med J 30(1):40-43, 2008). Hyperimmunoglobulin E syndrome is a primary immunodeficiency syndrome with multiple recurrent abscess formation and raised serum immunoglobulin E levels. We report a case of JDM with calcinosis cutis universalis with hyperimmunoglobulin E syndrome. With a previous similar case report (Min et al., Korean J Intern Med 14:95-98, 1999), this could well be a new sequence syndrome where abscesses are the trigger for the onset of JDM.

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We report a 12-year-old child with pseudohypoparathyroidism (PHP) whose mother had pseudopseudohypoparathyroidism. The child had low serum calcium, high phosphorous and high parathormone (PTH) levels. PHP occurs due to a defect in the guanine nucleotide binding protein (G protein). She also had hypothyroidism which is known to utilize the G protein pathway. She developed T 1 diabetes mellitus (T1DM) while under follow-up. This is arguably the first time T1DM has been reported associated with PHP.

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OBJECTIVE: Sex-ratio at birth in families with previous girls is worse than those with a boy. Our aim was to prospectively study in a large maternal and child unit sex-ratio against previous birth sex and use of traditional medicines for sex selection. MAIN OUTCOME MEASURES: Sex-ratio among mothers in families with a previous girl and in those with a previous boy, prevalence of indigenous medicine use and sex-ratio in those using medicines for sex selection. RESULTS: Overall there were 806 girls to 1000 boys. The sex-ratio was 720:1000 if there was one previous girl and 178:1000 if there were two previous girls. In second children of families with a previous boy 1017 girls were born per 1000 boys. Sex-ratio in those with one previous girl, who were taking traditional medicines for sex selection, was 928:1000. CONCLUSION: Evidence from the second children clearly shows the sex-ratio is being manipulated by human interventions. More mothers with previous girls tend to use traditional medicines for sex selection, in their subsequent pregnancies. Those taking such medication do not seem to be helped according to expectations. They seem to rely on this method and so are less likely use more definitive methods like sex selective abortions. This is the first such prospective investigation of sex ratio in second children looked at against the sex of previous children. More studies are needed to confirm the findings.

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