Hemolytic uremic syndrome (HUS)

Ardissino G, et al. Early volume expansion and outcomes of hemolytic uremic syndrome. Pediatrics. 2016 Jan;137(1).

Patients with STEC-HUS had great benefit from early volume expansion. It is speculated that early and generous fluid infusions can reduce thrombus formation and ischemic organ damage, thus having positive effects on both short- and long-term disease outcomes.

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Kappler S, et al. Thrombotic microangiopathies (TTP, HUS, HELLP). Emerg Med Clin North Am. 2014 Aug;32(3):649-71.

“Typical HUS is treated with supportive care, which includes blood transfusions when needed, judicious control of hypertension (preferably with nifedipine or nicardipine), careful maintenance of fluids and electrolytes, and hemodialysis when clinically indicated. [32] Dialysis was required in 63% of patients with HUS in one study. [33] In a meta-analysis of treatment modalities for typical HUS, there was no difference in mortality or clinical outcome when supportive care was compared with FFP infusion, anticoagulation medications, steroids, or a Shiga-toxin-binding agent. 9 With no proven benefit and a potential to worsen the disease process, antibiotics, narcotics, and antimotility agents should also be avoided in HUS. [31 36 37] PET therapy lacks compelling support in children with typical HUS and is controversial in adults. [5 30 38 39] Despite the lack of evidence, PET therapy may be considered when severe neurologic abnormalities are present. [13]”

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Altered mental status and delirium in children

Souganidis E, Grala M, Chinsky J. Six-year-old with altered mental status: no “LACk” of clues. Pediatr Emerg Care. 2015 Apr 14. [Epub ahead of print]

“The clinical approach to a child with altered mental status requires both a broad differential diagnosis as well as prompt efforts to resuscitate and stabilize the child. While the diagnostic work-up may require extensive laboratory testing and imaging, there should always be an initial focus on rapidly reversible causes, most notably hypoglycemia in this case. Although the differential diagnosis of hypoglycemia is in itself extensive and largely influenced by the age of the child, the ability to promptly administer glucose, as that was done with our patient, can be life-saving before the etiology of the hypoglycemic episode is identified.”

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Grover S, Kate N, Malhotra S, et al. Symptom profile of delirium in children and adolescent–does it differ from adults and elderly? Gen Hosp Psychiatry. 2012 Nov-Dec;34(6):626-32.

“The commonly observed symptoms in children and adolescents with delirium were disturbance in attention, orientation, sleep-wake cycle disturbances, fluctuation of symptoms, disturbance of short-term memory and motor agitation. The least commonly seen symptoms included delusions and motor retardation. Compared to adults, children and adolescents had lower frequency of long-term memory and visuospatial disturbances. Compared to the elderly, children and adolescents had higher frequency of lability of affect. For severity of symptoms, compared to adults, the children and adolescents had lower severity of sleep-wake disturbances, abnormality of thought, motor agitation, orientation, attention, short-term memory, long-term memory and visuospatial abilities. When compared to elderly patients, children and adolescents had higher severity of lability of affect and lower severity of language disturbances, short-term memory and visuospatial abilities.”

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Neutropenia (Non-chemotherapy-induced)

Lindqvist H, et al. Neutropenia in childhood: a 5-year experience at a tertiary center. Eur J Pediatr. 2015 Jun;174(6):801-7.

Clinical characteristics corroborated by laboratory investigations are essential to determine the etiology in cases of childhood neutropenia and the level of future health-care needs. Here the presentation, findings, and need of interventions in different types of neutropenia in children followed at our center from 2007 to 2012 were investigated retrospectively. Children with congenital and autoimmune neutropenia presented at a significantly younger age and with lower absolute neutrophil granulocyte counts than those with other types of neutropenia (p < 0.01-0.05). The duration of neutropenia, in case of remission, was shorter in post-infection and drug-induced cases compared to autoimmune and chronic idiopathic neutropenias (p = 0.001). Least affected from infections were children with ethnic and post-infection neutropenias compared to the others (p = 0.01-0.05). With the exception of congenital and autoimmune neutropenias, neutropenic children had few clinical infections and few hospital admissions even though the outpatient visit frequency was similar among the groups. A vast majority of the patients received no antibiotic prophylaxis.

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Walkovich K, Boxer LA. How to approach neutropenia in childhood. Pediatr Rev. 2013 Apr;34(4):173-84.

  1. Recognize patients who have concerning features of history, physical examination, or laboratory results that warrant further investigation for possible neutropenia or other immunodeficiency.
  2. Define mild, moderate, and severe neutropenia.
  3. Understand that neutropenia can arise from acquired or intrinsic conditions. Know which causes of neutropenia are most commonly encountered in childhood.

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Iron-deficiency anemia and Helicobacter pylori infections

Roma E, Miele E. Helicobacter pylori infection in pediatrics. Helicobacter. 2015 Sep;20 Suppl 1:47-53.

This review includes the main pediatric studies published from April 2014 to March 2015. The host response of Treg cells with increases in FOXP3 and TGF-β1 combined with a reduction in IFN-γ by Teff cells may contribute to Helicobacter pylori susceptibility in children. Genotypic variability in H. pylori strains influences the clinical manifestation of the infection. Helicobacter pylori infection is associated with variables indicative of a crowded environment and poor living conditions, while breast-feeding has a protective effect. Intrafamilial infection, especially from mother to children and from sibling to sibling, is the dominant transmission route. Studies showed conflicting results regarding the association between H. pylori infection and iron deficiency anemia. One study suggests that H. pylori eradication plays a role in the management of chronic immune thrombocytopenic purpura in H. pylori-infected children and adolescents. The prevalence of H. pylori was higher in chronic urticaria patients than in controls and, following H. pylori eradication, urticarial symptoms disappeared. An inverse relationship between H. pylori infection and allergic disease was reported. Antibiotic resistance and insufficient compliance to treatment limit the efficacy of eradication therapy.

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Queiroz DM, et al. Iron status and Helicobacter pylori infection in symptomatic children: an international multi-centered study. PLoS One. 2013 Jul 4;8(7):e68833.

“Conversely, in the United Kingdom cohort, the very low prevalence of H. pylori infection in an ethnically diverse group of children might explain the absence of association between the infection and iron deficiency/IDA parameters. A decreased prevalence of H. pylori infection associated with different generations of immigrants from developing to developed countries was similarly described by Tsai et al. [29] with Hispanics in the USA, likely reflecting a better standard of socioeconomic conditions. Thus, geographical variability among iron stores of the children may also explain the differences between the Latin American and UK cohorts we observed. Due to inadequate diet, children from developing countries could have a small iron reserve that contributes to the development of iron deficiency and IDA in the course of H. pylori infection.”

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Peritonitis in nephrotic syndrome patients

Teo S, Walker A, Steer A. Spontaneous bacterial peritonitis as a presenting feature of nephrotic syndrome. J Paediatr Child Health. 2013 Dec;49(12):1069-71.

Key Points

  1. In young children presenting with abdominal pain, clinicians must examine thoroughly to exclude peripheral oedema, routinely perform urinalysis to exclude heavy proteinuria and consider nephrotic syndrome as the primary diagnosis.
  2. In children with nephrotic syndrome who present with abdominal pain, spontaneous bacterial peritonitis should be considered as the most important differential diagnosis.
  3. Patients with nephrotic syndrome are at a higher risk of invasive bacterial infection, especially with Streptococcus pneumoniae, and specific strategies including pneumococcal vaccination and penicillin prophylaxis are important in preventing serious infection.

Ajayan P, et al. Clinical spectrum and predictive risk factors of major infections in hospitalized children with nephrotic syndrome. Indian Pediatr. 2013 Aug;50(8):779-81.

This observational study was conducted with the primary objective of studying the incidence of major infections in nephrotic syndrome (NS), while the secondary objectives were to evaluate the risk factors for and the etiological spectrum of major infections. Eighty six children up to 13 years of age fulfilling the International Study of Kidney Disease in Children (ISKDC) criteria for NS, who required 101 hospital admissions were recruited from November 2010 to July 2012. Major infections were defined as those that are disseminated, affecting deep organs, requiring hospitalization or potentially life-threatening. The incidence of major infections was 36.6%. Among the major infections, peritonitis and pneumonia together accounted for 72.9%, while urinary tract infections and cellulitis accounted for 16.2%. On logistic regression, severe ascites and more severe clinical types of NS independently predicted major infections, while serum cholesterol >400 mg/dL was the sole predictor of peritonitis.

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Use of steroids in treatment of Henoch–Schonlein purpura (HSP)

Hahn D, et al. Interventions for preventing and treating kidney disease in Henoch-Schönlein Purpura (HSP). Cochrane Database Syst Rev. 2015 Aug 7;(8):CD005128.

There are no substantial changes in conclusions from this update compared with the initial review. From generally low quality evidence, we found no evidence of benefit from RCTs for the use of prednisone or antiplatelet agents to prevent persistent kidney disease in children with HSP. Though heparin appeared effective, this potentially dangerous therapy is not justified to prevent serious kidney disease when fewer than 2% of children with HSP develop severe kidney disease. No evidence of benefit has been found for cyclophosphamide treatment in children or adults with HSP and severe kidney disease. Because of small patient numbers and events leading to imprecision in results, it remains unclear whether cyclosporin and mycophenolate mofetil have any roles in the treatment of children with HSP and severe kidney disease.

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Dudley J, et al. Randomised, double-blind, placebo-controlled trial to determine whether steroids reduce the incidence and severity of nephropathy in Henoch-Schonlein Purpura (HSP). Arch Dis Child. 2013 Oct;98(10):756-63.

“This is the largest trial of the role of steroids in children with HSP. We found no evidence to suggest that early treatment with prednisolone reduces the prevalence of proteinuria 12 months after disease onset in children with HSP.”

Comment in: Prevention of renal disease in Henoch-Schonlein purpura: clear evidence against steroids.

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Somnambulism (sleepwalking)

Sleepwalking

 

 

 

 

 

 

 

Petit D, et al. Childhood Sleepwalking and Sleep Terrors: A Longitudinal Study of Prevalence and Familial Aggregation. JAMA Pediatr. 2015 Jul;169(7):653-8.

At a Glance:

  • This large prospective cohort study examines the prevalence of sleep terrors and sleepwalking and association of these with parental history.
  • The peak of prevalence was observed at age 11/2 years for sleep terrors (34.4%) and at age 10 years for sleepwalking (13.4%).
  • As many as one-third of children who had early childhood sleep terrors developed sleepwalking later in childhood.
  • The prevalence of childhood sleepwalking increases with parental history of sleepwalking: 22.5% for children without parental history, 47.4% for children with 1 parent with a history of sleepwalking, and 61.5% for children with both parents with a history of sleepwalking.
  • These findings point to a strong genetic influence on sleepwalking and, to a lesser degree, sleep terrors.

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Neonatal abstinence syndrome

McQueen K, Murphy-Oikonen J. Neonatal Abstinence Syndrome. N Engl J Med. 2016
Dec 22;375(25):2468-2479.

The purpose of this review is to summarize the current literature on the neonatal abstinence syndrome, including clinical characteristics, prevention, identification, and treatment. Approaches to care that recognize the importance of the infant–mother dyad are emphasized when possible.

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Practice Based Learning: Utility of Ranitidine and PPIs in infantile GERD

Zev Cohen MD

 

 

 

 

Presented by Zev Cohen MD (PGY2)

Clinical Questions:

  • What is the efficacy of proton-pump inhibitors (PPIs)/ranitidine for infantile gastroesophageal reflux (GERD)?
  • Is a PPI more effective than ranitidine?
  • What is the durability of ranitidine?

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