Twenty years' experience of steroids in infantile hemangioma—a developing country's perspective

Background

Hemangioma is a common vascular tumor. Though it involutes spontaneously, results are unpredictable. Steroid therapy is an effective mode of its regression. We present our experience of largest series and possible recommendations for treatment.

Materials and methods

A total of 2398 patients were treated during the study period of 20 years. They were given oral prednisolone, intralesional triamcinolone, or combination of both as per the protocol and followed for the response. Response to the treatment was graded as excellent, good, poor, or no response.

Results

The male-to-female ratio was 1:2.3. In 81% of patients, hemangioma was noticed within first month of life. The commonest site of involvement was head and neck (57%). The commonest clinical presentation was discoloration and swelling. Mean age and size were 8.43 ± 7.04 months and 23.64 ± 20.13 cm². Response rate was highest for superficial type using any modality of treatment. Patients younger than 1 year showed better response (90.3%) in comparison with children older than 1 year (80.8%). The specific complications occurring were infections in 249 (12.4%), cushingoid facies and growth delay in 62 (3.1%), and hypertension in 51 (2.5%) patients.

Conclusion

Steroid therapy either oral or intralesional as per the requirement is an easy and safe modality. Results are good to satisfactory in most patients. The complications are minimal. If treatment is needed, it should be used as a first-line therapy, especially when cost is an important concern.     

Matrix metalloproteinases in ascending aortic aneurysms: bicuspid versus trileaflet aortic valves

BACKGROUND: Abnormal matrix metalloproteinase (MMP) and tissue inhibitor of metalloproteinase (TIMP) expression contributes to the development of abdominal aortic aneurysms. Recent data suggest that MMP-2 and MMP-9 may also play a role in thoracic aortic disease. We sought to determine (1) whether ascending aortic aneurysms are associated with increased MMP expression and (2) whether aortic inflammation and MMP expression differ between patients with congenital bicuspid aortic valves (BAVs) and those with trileaflet aortic valves (TAVs). MATERIALS AND METHODS: Samples of ascending aortic aneurysms were obtained from 29 patients; 14 patients had BAVs and 15 had TAVs. Control ascending aorta was obtained from 14 organ donors or heart transplant recipients. Aortic histology and immunohistochemistry were performed to evaluate elastin degradation, inflammatory changes, and MMP-2 and MMP-9 expression. Aortic levels of MMP-2, MMP-9, TIMP-1, and TIMP-2 were measured using ELISA. RESULTS: Aneurysms in the TAV patients exhibited marked inflammation, high CD68 expression, diminished elastin content, increased MMP-9 expression, and normal MMP-2 levels. In contrast, BAV aneurysms were characterized by a relative lack of inflammation, preservation of elastin content, normal MMP-9 levels, and elevated MMP-2 expression. TIMP-1 and TIMP-2 levels were not significantly different among the three groups. CONCLUSIONS: Ascending aortic aneurysms exhibited increased MMP expression. The pattern of MMP expression and the degree of inflammation, however, differed between aneurysms associated with BAVs and those with TAVs. Variations in the molecular mechanisms underlying different types of thoracic aortic aneurysms warrant further investigation.     

Self-reported hypoglycaemia in insulin-treated patients with diabetes mellitus: results from the Singapore cohort of the International Operations Hypoglycaemia Assessment Tool study

INTRODUCTIONHypoglycaemia constitutes a significant barrier to achieving glycaemic control with insulin in both Type 1 (T1DM) and Type 2 diabetes mellitus (T2DM). The International Operations Hypoglycaemia Assessment Tool (IO HAT) study was designed to determine the incidence of hypoglycaemia in insulin-treated patients with T1DM and T2DM.METHODSThe IO HAT study retrospectively and prospectively assessed the incidence of hypoglycaemia in patients with insulin-treated diabetes mellitus in nine countries. This sub-analysis included patients from Singapore with T1DM or T2DM who were aged ≥ 21 years and had completed two self-assessment questionnaires (SAQ1 and SAQ2).RESULTSOf the 50 T1DM and 320 T2DM patients who completed the SAQ1, 39 T1DM and 265 T2DM patients completed SAQ2; 100% and 90.9%, respectively, experienced at least one hypoglycaemic event prospectively. The incidence rates of any hypoglycaemia were 49.5 events per patient-year (EPPY) and 16.1 EPPY for T1DM and T2DM patients, respectively, in the four-week prospective period. Hypoglycaemia rate did not differ in terms of glycated haemoglobin level. The vast majority of T1DM or T2DM patients (92.0% and 90.7%, respectively) knew the overall definition of hypoglycaemia before study participation, although over half of the patients (T1DM 54.0%, T2DM 51.9%) defined hypoglycaemia based only on symptoms.CONCLUSIONHigh proportions of insulin-treated patients with diabetes mellitus in Singapore reported hypoglycaemic events prospectively, showing that they had underreported hypoglycaemic episodes retrospectively. Patient education can help in improving hypoglycaemia awareness and its management in the region.     

One-year-old male with accelerated growth and development

A 1-year-old male child with isosexual central (gonadotropin-dependent) precocious puberty caused by hypothalamic hamartoma is reported. Details of the diagnosis based solely on neuromaging characteristics, and satisfactory results of medical treatment with gonadotropin releasing hormone agonist analogues, are highlighted.     

Clinical and genetic features of variegate porphyria in a Chinese patient

Acute porphyria is rare in orientals. We describe a Chinese woman with recurrent generalised tonic-clonic seizures and abdominal pain. Genomic DNA studies identified a heterozygous base substitution from guanine to adenine at nucleotide position 503, resulting in substitution of arginine by histidine at position 168 of the protein (R168H). This genetic abnormality is similar to the mutation reported in Caucasians with variegate porphyria. To the best of our knowledge, this is the first report in the English literature a Chinese patient with variegate porphyria with an identifiable mutation. A brief review of porphyria is presented.     

Epidemiology and treatment of painful procedures in neonates in intensive care units

Ricardo Carbajal, MD, PhD; André Rousset, MD; Claude Danan, MD; Sarah Coquery, MD; Paul Nolent, MD; Sarah Ducrocq, MD; Carole Saizou, MD; Alexandre Lapillonne, MD, PhD; Michèle Granier, MD; Philippe Durand, MD; Richard Lenclen, MD; Anne Coursol, MD; Philippe Hubert, MD, PhD; Laure de Saint Blanquat, MD; Pierre-Yves Boëlle, PhD; Daniel Annequin, MD; Patricia Cimerman, RN; K. J. S. Anand, MBBS, DPhil; Gérard Bréart, MD, PhD

JAMA. 2008;300(1):60-70.

Context  Effective strategies to improve pain management in neonates require a clear understanding of the epidemiology and management of procedural pain.

Objective  To report epidemiological data on neonatal pain collected from a geographically defined region, based on direct bedside observation of neonates.

Design, Setting, and Patients  Between September 2005 and January 2006, data on all painful and stressful procedures and corresponding analgesic therapy from the first 14 days of admission were prospectively collected within a 6-week period from 430 neonates admitted to tertiary care centers in the Paris region of France (11.3 millions inhabitants) for the Epidemiology of Procedural Pain in Neonates (EPIPPAIN) study.

Main Outcome Measure  Number of procedures considered painful or stressful by health personnel and corresponding analgesic therapy.

Results  The mean (SD) gestational age and intensive care unit stay were 33.0 (4.6) weeks and 8.4 (4.6) calendar days, respectively. Neonates experienced 60 969 first-attempt procedures, with 42 413 (69.6%) painful and 18 556 (30.4%) stressful procedures; 11 546 supplemental attempts were performed during procedures including 10 366 (89.8%) for painful and 1180 (10.2%) for stressful procedures. Each neonate experienced a median of 115 (range, 4-613) procedures during the study period and 16 (range, 0-62) procedures per day of hospitalization. Of these, each neonate experienced a median of 75 (range, 3-364) painful procedures during the study period and 10 (range, 0-51) painful procedures per day of hospitalization. Of the 42 413 painful procedures, 2.1% were performed with pharmacological-only therapy; 18.2% with nonpharmacological-only interventions, 20.8% with pharmacological, nonpharmacological, or both types of therapy; and 79.2% without specific analgesia, and 34.2% were performed while the neonate was receiving concurrent analgesic or anesthetic infusions for other reasons. Prematurity, category of procedure, parental presence, surgery, daytime, and day of procedure after the first day of admission were associated with greater use of specific preprocedural analgesia, whereas mechanical ventilation, noninvasive ventilation and administration of nonspecific concurrent analgesia were associated with lower use of specific preprocedural analgesia.

Conclusion  During neonatal intensive care in the Paris region, large numbers of painful and stressful procedures were performed, the majority of which were not accompanied by analgesia.

     

Effects of reserpine on blood-pressure responses evoked from the hypothalamus

Different areas of the hypothalamus were stimulated by a stereotaxic technique before and after reserpine in unanaesthetized cats. Reserpine in a dose of 1 mg./kg. body weight increased the threshold of stimulation of hypothalamic areas in cats giving an initial pressor response. It decreased the threshold of stimulation of areas of the hypothalamus in animals giving an initial depressor response. Reserpine thus appeared to affect the hypothalamus directly in two ways. It depressed the sympathetic centres in the diencephalon and facilitated the parasympathetic ones.