Background: Status dystonicus is a life-threatening, underrecognized movement disorder emergency. We aimed to ascertain the etiology, clinical presentation, complications, and outcomes of status dystonicus in children and reviewed the literature for similar studies. Methods: Records of all children aged <14 years admitted to a single center with status dystonicus between 2014 and 2018 were reviewed. Results: Twenty-four children (75% male) were identified with status dystonicus. The annual incidence rate was 0.05 per 1000 new admissions <12 years of age. The mean age at presentation was 6.3 ± 3.6 years. Median duration of hospital stay was 10.5 days (interquartile range 5-21.7). The severity of dystonia at presentation was grade 3 (n = 9; 37.5%) and 4 (n = 9; 37.5%). The most common triggering factor was intercurrent illness/infection (n = 18; 75%). The most common underlying etiologies were cerebral palsy (n = 8; 33.3%), complicated tubercular meningitis (n = 3; 12.5%), and mitochondrial disorders (n = 3; 12.5%). Basal ganglia involvement was seen in 15 cases (62.5%). Respiratory and/or bulbar compromise (n = 20; 83.3%) and rhabdomyolysis (n = 15; 62.5%) were most commonly seen. Oral trihexyphenidyl (96%) followed by oral or intravenous diazepam (71%), oral baclofen (67%), and midazolam infusion (54%) were the most common drugs used. Clonidine was used in 33% cases, without any significant side effects. Three children died owing to refractory status dystonicus and its complications; the mortality rate was 12.5%. Conclusion Status dystonicus is a neurologic emergency in children with severe dystonia, with significant complications and a high mortality rate. Static and acquired disorders are more common than heredo-familial causes. Identification and treatment of infection in children is important as the majority of cases are triggered by an intercurrent infection.
Infection is an important complication of childhood nephrotic syndrome (NS) and spontaneous bacterial peritonitis (SBP) is a frequently encountered one. We present a 7-year-old boy with NS who had decreased urine output, generalized body swelling, and abdominal pain. Urine analysis showed proteinuria of 50 mg/m2/d. Ascitic tap showed total leukocyte count of 100 cells/mm3, sugar of 67 mg/dL, and protein of 1.1 g/dL. Gram stain revealed gram-negative bacilli with pus cells and culture grown Leclercia adecarboxylata (LAD). LAD was identified using matrix-assisted laser desorption ionization-time of flight mass spectrometry (MALDI-TOF MS) with an identification score of 2.0. The organism showed good susceptibility to common antibiotics. The boy had no direct contact with livestock and the source of infection remains speculative. Devitalized skin because of massive edema seems to be the most plausible site of entry for the organism. Our patient was started on ceftriaxone and improved. LAD is a rare opportunistic pathogen, which belongs to Enterobacteriaceae and usually causes soft tissue infections. As far as we know, this is the first case where it has caused peritonitis in a child with NS. We also reviewed other pediatric cases.
factors within that particular home and dynamic. However, due to the small numbers of cases of SIDS in the time frame reviewed it was impossible to determine whether the inclusion of social care involvement to the SBS would improve the statistical model overall.
Background Preterm infants may be more vulnerable to fractures due to physiological, metabolic and environmental factors, but an increased risk of fractures up to the age of 2 is unproven. The diagnosis of child abuse is one of exclusion and otherwise unexplained fractures in infants and young children may be erroneously attributed to premature birth despite the lack of evidence. The dilemma is complicated by reports that preterm children are more likely to be subjected to abuse as compared to term children. Epidemiological and clinical data comparing fractures in both preterm and term children could help experts form an opinion on the possibility of child abuse. Objectives To ascertain the rate of fractures, any differences in clinical presentation between preterm and term populations in the first 3 years of life and describe any differences in fracture patterns with an emphasis on fractures specific for abuse (rib and metaphyseal). Methods A retrospective study was conducted of children (term or preterm) born in the neonatal department of [screened] and subsequently attending the Emergency Department at [screened] with a suspected fracture within a 10-year period. We excluded any child who returned with the same injury, with known metabolic bone disease, with any disease or condition known to reduce bone density, who received any medication known to affect Vitamin D metabolism within 3 months of enrolment or who had fractures post-surgery/resuscitation. Variables such as the number of fractures sustained each year, age of presentation to the Emergency Department and mechanism of injury were compared between the preterm and term groups using statistical analysis (c2 and Fisher exact test for categorical variables and Student's t-test for continuous variables). Simple linear regression was performed on the total number of fractures sustained by age 3. Results 3,737 children were born and 2,533 attended ED during the study period, of which 79 attended with fractures. 44 children were included. Of these, none were born extremely preterm, 24 (55%) were preterm, and 20 (45%) were born at term. Mean gestational ages of the preterm and term groups were 32 weeks 3 days and 39 weeks 6 days, respectively. There were no extremely low birth weight or very low birth weight children. There was no significant difference in the number of fractures sustained yearly, the age of presentation to the Emergency Department or the site of fracture between preterm and term groups. Linear regression showed that the total number of fractures sustained by age 3 years was unrelated to prematurity status, gender or birth weight category. Conclusions Our data failed to show any association between prematurity and risk of childhood fractures up to the age of 3 years. Clinical presentation, site and types of fractures sustained by premature infants were not different from the term cohort. There were no fractures typical of abuse presenting over the 10-year study period, which suggests they are an uncommon finding in preterm children up to the ...
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