Infantile Parotid Hemangioma With Diagnostic Dilemma: A Case Report.

INTRODUCTION: Salivary gland tumors are uncommon in children; hemangiomas are one of them. We report a case of infantile hemangioma of the parotid gland which posed a diagnostic dilemma. PATIENTS AND METHODS: Four-month-old infant presented with swelling at the parotid gland region, which was progressively increasing. There was a diagnostic dilemma with initial misdiagnosis as acute parotitis. Imaging studies with ultrasonography and CT with contrast were requested. RESULTS: Imaging studies revealed hyperintense lobulated mass suggestive of parotid hemangioma. Vascular consultation recommended conservative management with follow-up after 3 months. On follow-up, there was an increase in mass size, and propranolol was started. Swelling showed a good response on the next follow-up visit and is still on medical management. CONCLUSION: Parotid hemangiomas frequently pose a diagnostic dilemma, but the typical noninflammatory nature of the swelling and radiological evaluation confirms the diagnosis.

Sjogren-Larsson Syndrome: A case series of five members from an extended family with a novel mutation.

BACKGROUNDD: Sjogren-Larsson syndrome (SLS) is a rare autosomal recessive disorder, characterized by a triad of spastic tetraplegia or diplegia, congenital ichthyosis, and intellectual disability. METHODS: We report a seven-years-old female born to consanguineous parents who presented with erythematous dry scaly skin all over the body sparing the face, without collodion membrane which started since birth. There were associated with global developmental delay and seizure disorder. SLS was suspected and hence sequence analysis of the ALDH3A2 gene by next-generation sequencing was performed for the patient. RESULTS: A novel nucleotide exchange in homozygous state at position c.1320 in exon 9 of the ALDH3A2 gene (c.1320T>A), leading to a stop of the protein sequence (p.Tyr440) was detected in the patient. Genetic testing of the patient's extended family revealed another four affected family members with the same mutation. CONCLUSIONS: SLS should be suspected in any patient with a triad of ichthyosis, intellectual disability and spastic di/tetraplegia. Molecular genetic testing of the ALDH3A2 gene should be performed to confirm the diagnosis. Extended family screening is highly recommended.

Helicobacter pylori seropositivity protects against childhood asthma and inversely correlates to its clinical and functional severity.

BACKGROUND: In recent years, the prevalence of asthma has risen in developed countries, and its extent related to a change in our indigenous microbiota. Helicobacter pylori disappearance across the population represents a fundamental change in our human microbiota and has preceded the rise in asthma prevalence. OBJECTIVE: To assess the relationship between childhood asthma and Helicobacter pylori infection. METHODS: Quantitative determination of Helicobacter pylori IgG among 90 asthmatic children and 90 - age and gender - matched non-atopic, non-asthmatic healthy children was performed using ELISA in serum of all participants. RESULTS: Helicobacter pylori IgG seropositivity was found in 25.6% of asthmatics compared to 44.4% of controls. Asthmatics showed lower median Helicobacter pylori IgG titre compared to healthy controls. We also detected a significant inverse relationship between Helicobacter pylori IgG titre and asthma severity. CONCLUSION: Helicobacter pylori seropositivity protects against childhood asthma and inversely correlates to its clinical and functional severity.

Prognostic significance of central venous-to-arterial carbon dioxide difference during the first 24 hours of septic shock in patients with and without impaired cardiac function.

OBJECTIVE: To investigate the prognostic significance of central venous-to-arterial carbon dioxide difference (cv-art CO 2 gap) during septic shock in patients with and without impaired cardiac function. METHODS: We performed a prospective cohort study in 10 French intensive care units. Patients suffering from septic shock were assigned to the impaired cardiac function group ('cardiac group', n =123) if they had atrial fibrillation (AF) and/or left ventricular ejection fraction (LVEF) <50% at study entry and to the non-cardiac group ( n =240) otherwise. RESULTS: Central venous and arterial blood gases were sampled every 6 h during the first 24 h to calculate cv-art CO 2 gap. Patients in the cardiac group had a higher cv-art CO 2 gap [at study entry and 6 and 12 h (all P <0.02)] than the non-cardiac group. Patients in the cardiac group with a cv-art CO 2 gap >0.9 kPa at 12 h had a higher risk of day 28 mortality (hazard ratio=3.18; P =0.0049). Among the 59 patients in the cardiac group with mean arterial pressure (MAP) ≥65 mm Hg, central venous pressure (CVP) ≥8 mm Hg and central venous oxygen saturation (ScvO 2 ) ≥70% at 12 h, those with a high cv-art CO 2 gap (>0.9 kPa; n =19) had a higher day 28 mortality (37% vs. 13%; P =0.042). In the non-cardiac group, a high cv-art CO 2 gap was not linked to a higher risk of day 28 death, whatever the threshold value of the cv-art CO 2 gap. CONCLUSION: Patients with septic shock and with AF and/or low LVEF were more prone to a persistent high cv-art CO 2 gap, even when initial resuscitation succeeded in normalizing MAP, CVP, and ScvO 2 . In these patients, a persistent high cv-art CO 2 gap at 12 h was significantly associated with higher day 28 mortality.

Change in end-tidal carbon dioxide outperforms other surrogates for change in cardiac output during fluid challenge.

Background: During fluid challenge, volume expansion (VE)-induced increase in cardiac output (Δ VE CO) is seldom measured. Methods: In patients with shock undergoing strictly controlled mechanical ventilation and receiving VE, we assessed minimally invasive surrogates for Δ VE CO (by transthoracic echocardiography): fluid-induced increases in end-tidal carbon dioxide (Δ VE E'CO2 ); pulse (Δ VE PP), systolic (Δ VE SBP), and mean systemic blood pressure (Δ VE MBP); and femoral artery Doppler flow (Δ VE FemFlow). In the absence of arrhythmia, fluid-induced decrease in heart rate (Δ VE HR) and in pulse pressure respiratory variation (Δ VE PPV) were also evaluated. Areas under the receiver operating characteristic curves (AUC ROC s) reflect the ability to identify a response to VE (Δ VE CO ≥15%). Results: In 86 patients, Δ VE E'CO2 had an AUC ROC =0.82 [interquartile range 0.73-0.90], significantly higher than the AUC ROC for Δ VE PP, Δ VE SBP, Δ VE MBP, and Δ VE FemFlow (AUC ROC =0.61-0.65, all P <0.05). A value of Δ VE E'CO2 >1 mm Hg (>0.13 kPa) had good positive (5.0 [2.6-9.8]) and fair negative (0.29 [0.2-0.5]) likelihood ratios. The 16 patients with arrhythmia had similar relationships between Δ VE E'CO2 and Δ VE CO to patients with regular rhythm ( r 2 =0.23 in both subgroups). In 60 patients with no arrhythmia, Δ VE E'CO2 (AUC ROC =0.84 [0.72-0.92]) outperformed Δ VE HR (AUC ROC =0.52 [0.39-0.66], P <0.05) and tended to outperform Δ VE PPV (AUC ROC =0.73 [0.60-0.84], P =0.21). In the 45 patients with no arrhythmia and receiving ventilation with tidal volume <8 ml kg -1 , Δ VE E'CO2 performed better than Δ VE PPV, with AUC ROC =0.86 [0.72-0.95] vs 0.66 [0.49-0.80], P =0.02. Conclusions: Δ VE E'CO2 outperformed Δ VE PP, Δ VE SBP, Δ VE MBP, Δ VE FemFlow, and Δ VE HR and, during protective ventilation, arrhythmia, or both, it also outperformed Δ VE PPV. A value of Δ VE E'CO2 >1 mm Hg (>0.13 kPa) indicated a likely response to VE.

Serum and nasal lavage fluid Clara cell protein decreases in children with allergic rhinitis.

BACKGROUND: Allergic rhinitis is among the most common chronic disorders of childhood with prevalence of up to 40% in children. Clara cell secretory protein (CCSP) is secreted by Clara cells in the lining fluid of airways. It has an immune-modulatory and anti-inflammatory activity. AIM OF WORK: Study aimed at evaluating CCSP as a biomarker in serum and nasal lavage fluid of children with allergic rhinitis. METHODS: A case-control study was conducted on sera and nasal lavage fluid samples from 15 children with allergic rhinitis and 15 healthy children as a control group. RESULTS: Children with allergic rhinitis had a male to female ratio 2 to 1, with a mean age of 9.47±2.75 years, while among the healthy group, six were males and nine were females, with a mean age of 8.63±2.28 years. Rhinorrhea and nasal obstruction were the most frequent symptoms (100%) followed by itching (93.3%) then sneezing (73.3%). Among allergic rhinitis patients serum CCSP mean±SD was 2.03±0.59µg/l; it was reliable to predict allergic rhinitis (P<0.0001); while nasal lavage CCSP mean±SD was 12.73±8.25µg/l and it was not reliable to predict allergic rhinitis. Its best cut-off value was 3.75µg/l with a sensitivity of 100%, specificity 80%, with a diagnostic accuracy of 90%. CONCLUSION: Clara cell secretory protein is a new peripheral sensitive marker of airway injury. Furthermore, serum CCSP level is a predictor of allergic rhinitis but not nasal lavage fluid CCSP.

Basic fibroblast growth factor-like substance in nuclei of male germ cells undergoing meiosis.

The presence of a basic fibroblast growth factor-like immunoreactive substance was demonstrated in the nuclei of germ cells at stages from spermatocyte to spermatid in adult rat testis by using immunohistochemistry with an antibody raised against a synthetic peptide corresponding to residues 1-10 of bovine basic fibroblast growth factor [1-146]. The fluorescence was very weak in the nuclei and cytoplasm of spermatogonia, Sertoli cells, and most of the interstitial compartments, except for capillary endothelial cells. This is the first study to demonstrate the presence of basic fibroblast growth factor-like immunoreactive material in the nuclei of haploid cells in vivo.

Sensorineural hearing loss in sickle cell crisis.

The present investigation examines the effects of sickle cell crisis on peripheral auditory function and auditory brainstem response (ABR). The study includes 10 homozygous sickle cell anemia patients with documented sickle cell (SS) hemoglobin. Testing was performed during the crisis and 1 month after it subsided. Unilateral high frequency hearing loss was found in three patients but persisted in only one of them. Abnormal ABR tracings occurred in four patients but reversed to normal in one of them. The study suggests a trend toward increased incidence of sensorineural hearing loss (SNHL) and ABR changes as frequency and severity of crisis episodes increases. The relationship between SNHL and ABR is analyzed and discussed.

Hearing and middle ear function in rheumatoid arthritis.

The hearing and middle ear function in 68 patients with rheumatoid arthritis were tested. Reduced middle ear compliance, presumably due to stiffening of the tympanossicular chain, was noted in 48 patients (70.50%). Conductive hearing loss and absence of the stapedius reflex, were recorded in only one patient (1.40%). In 20 patients (29.40%) a moderate (20-30 dB) high frequency sensorineural hearing loss was found. The results of loudness balance and tone decay tests and the speech discrimination scores suggested cochlear pathology in some cases. However the multiplicity of factors that might be involved in sensorineural hearing loss, and the absence of a significant relation between such a loss and the activity of the disease or drugs used in its treatment makes the postulation of a plausible explanation a difficult, still unfinished task.

Broad spectrum antifungal agents in otomycosis.

The in vitro inhibitory activities of four currently used antimycotic agents (clotrimazole, econazole, miconazole and cyclopirox-olamine) against 304 fungal isolates comprising 51 species from 14 genera of moulds and yeasts, using a serial dilution procedure, were studied. Clotrimazole and econazole were found to have a grossly similar broad-spectrum antifungal activity, inhibiting all the tested yeasts and moulds at a concentration ranging from 0.1-4 micrograms/ml. At this range miconazole inhibited 90 per cent of the strains and cyclopirox-olamine inhibited 57 per cent only and thus they were less effective. Econazole 1 per cent solution was very effective in vivo in the treatment of otomycosis within 1-3 weeks. The drug was well tolerated, with no side-effects. Owing to the high broad-spectrum antifungal activity of clotrimazole and econazole, they should be the treatment of choice in otomycosis and can be used safely as otic drops.

Pneumatic nasal catheters: advantages and drawbacks.

The authors present their experience with pneumatic nasal catheters in cases of epistaxis. Among the advantages of this method over tamponade with petroleum jelly-covered gauze are: Effective tamponade without causing hypoxia, carbon dioxide retention or additional loading of the cardiopulmonary system; Less need for sedation; and Easy application and removal. Conversely, its most important adverse effect is the possibility of pressure necrosis of the nasal mucosa and nasal septum. This can be greatly reduced by considering a few technical recommendations.

Treatment of auricular hematoma using button technique.

10 male patients suffering from auricular hematoma were treated by evacuation of the hematoma followed by suturing two buttons to the ear using through-and-through staying silk sutures, leaving them attached for 1 week. Not a single case of recurrence has been encountered as this technique eliminates the dead space and does not allow reaccumulation of the hematoma.

Amyloid deposits in heart valves.

Localized amyloid deposits were found in 44% of the surgically removed heart valves. They are recognized only in severely destructed valves and the manner of the deposition closely resembles that of aortic intimal amyloid. Although the amyloid fibril protein of the chronically diseased valves is still unknown, it may be neither protein AA nor AL, and another type of amyloid is suggested.