Topical nitroglycerin and pain in purpura fulminans

Topical nitroglycerin has been previously described as an adjunctive therapy to increase perfusion to areas of purpura affected in purpura fulminans. We report a case of purpura fulminans in which topical nitroglycerin was found to provide analgesia after its application to purpuric lesions. The broader role for the use of topical nitroglycerin in pain management deserves further study and evaluation.     

Meningococcal purpura fulminans: untoward result of genetic polymorphism?]

Despite significant progress in intensive care medicine, the mortality of septic shock has not changed in recent years. Early recognition of subtle signs in favor of meningococcal sepsis, early antibiotic treatment, and aggressive hemodynamic support remains the cornerstone of therapy of severe meningococcal shock in children. Recent work has emphasized the role of genetic polymorphisms in various systems to explain the most severe cases: anti-inflammatory cytokine profile IL-10/TNF-alpha, elevated levels of plasminogen activator inhibitor type-1, variants of the gene for mannose-binding lectin complement pathway. This may explain the disillusionment of pediatric intensivists, and the general failure of immunotherapy for sepsis. Reasonable hope lies upon new meningococcal vaccines.     

Wheezing in school children is not always asthma

Our objective was to study whether children with reported asthma differed from children with wheeze but without asthma, and from children with neither asthma nor wheeze, regarding lung function, bronchial hyper‐responsiveness (BHR) using methacholine inhalation, exercise‐induced bronchoconstriction (EIB), and skin prick test (SPT) reactivity. School children (n=2188), enrolled in a survey of asthma, were classified into three mutually exclusive groups by parental report of: asthma, wheeze, and no asthma/no wheeze. A random sample of 80 children in each group was tested (n=240). Among asthmatics, 68% (95% confidence interval (CI), 57–79) had a BHR (measured as PD₂₀ forced expiratory volume in 1 s (FEV₁) ≤ 8.16 μmol using methacholine) compared to 31% (CI 20–42%) and 30% (CI 19–40%) in the wheeze and no asthma/no wheeze groups. The dose–response slope (DRS) confirmed the PD₂₀ data and distinguished equally between groups. EIB (≥10% fall in FEV₁) was more frequent (40%, CI 29–52%) among asthmatics than among children with wheeze (12%, CI 4–19%) and no asthma/no wheeze (7%, CI 1–13%). The prevalence of at least one positive SPT was twice as high in the asthma group (58%, 47–69%) than in the wheeze (27%, CI 16–37%) and the no asthma/no wheeze (25%, 15–35%) groups. These results indicate that children with asthma differ from children with wheeze and children with no asthma/no wheeze regarding lung function, BHR, EIB, and SPT reactivity. Children with wheeze are more similar to children with no asthma/no wheeze with respect to these parameters.     

Bruising: when it is spontaneous and not idiopathic thrombocytopenia purpura

Bruising is a presentation that often causes concern. There are many causes of bruising in children, including non-accidental injury, which must be excluded. We report a case of a 22-month-old boy where all the common diagnoses were excluded. We highlight the need to be aware of transient acquired inhibitors of coagulation that can cause spontaneous bleeding.     

Recombinant human activated protein C in the treatment of children with meningococcal purpura fulminans

Meningococcal purpura fulminans (MPF) produces high mortality and morbidity, despite appropriate standard therapy. Administration of recombinant human activated protein C (rhAPC) has been successfully applied in adults with MPF and pediatric studies are under way. We report three pediatric patients with MPF treated with rhAPC as compassionate therapy. In two of these patients, positive clinical and laboratory effects were observed and both children achieved full recovery. The remaining patient died after 36 hours from refractory multiorgan failure. No rhAPC-related adverse effects were detected. The reported cases highlight the usefulness of rhAPC in children with MPF at least as a rescue compassionate treatment. Further clinical trials are needed to better delineate its efficacy and administration schedule in children.     

Partial growth hormone insensitivity--idiopathic short stature is not always idiopathic

Heterozygous growth hormone receptor (GHR) gene defects are not a common cause of idiopathic short stature. Although some of these GHR mutations may result in relative insensitivity to growth hormone (GH) in other studies, obligate heterozygotes did not present any clinical manifestations. Although patients with GH insensitivity and elevated GH binding protein (GHBP) levels have been described, it may be a reasonable approach to screen children who have growth failure, low levels of insulin-like growth factor-I (IGF-I) and IGF-binding protein-3, and low levels of GHBP. Whether the sensitivity of this screening approach can be increased by administering pharmacological doses of GH for a few days and measuring the resultant increase in serum IGF-I concentration remains to be determined by ongoing studies.     

Torticollis: not always the usual suspects

We describe the clinical presentation, radiographic findings, management, and outcome of nontraumatic spinal epidural hematoma in a 10-month-old male infant with severe hemophilia (<1% activity). This patient presented with torticollis, and the differential diagnosis included intramuscular hemorrhage, retropharyngeal abscess, muscle spasm, and epidural hematoma. A computed tomography scan revealed extensive spinal epidural hematoma from C1-L4. Because of prompt diagnosis, this infant was able to be managed conservatively with factor VIII and did not require surgical intervention. Unlike other cases previously published, this case demonstrates how prompt recognition, diagnosis, and treatment can prevent the development of neurological deficits.     

Partial growth hormone insensitivity - idiopathic short stature is not always idiopathic

Saenger P. Partial growth hormone insensitivity - idiopathic short stature is not always idiopathic. Acta Pædiatr 1999; Suppl 428: 194–8. Stockholm. ISSN 0803–5326
Heterozygous growth hormone receptor (GHR) gene defects are not a common cause of idiopathic short stature. Although some of these GHR mutations may result in relative insensitivity to growth hormone (GH) in other studies, obligate heterozygotes did not present any clinical manifestations. Although patients with GH insensitivity and elevated GH binding protein (GHBP) levels have been described, it may be a reasonable approach to screen children who have growth failure, low levels of insulin-like growth factor-I (IGF-I) and IGF-binding protein-3, and low levels of GHBP. Whether the sensitivity of this screening approach can be increased by administering pharmacological doses of GH for a few days and measuring the resultant increase in serum IGF-I concentration remains to be determined by ongoing studies. □ Growth hormone insensitivity, growth hormone receptor, short stature