Objective: To compare the results of auditory steady-state response (ASSR) and click auditory brainstem response (click ABR) among infants and young children tested at the Ear Unit of a Tertiary General Hospital. Methods: Design: Cross-sectional Study Setting: Tertiary General Hospital Population: Within-subject comparisons of click auditory brainstem response (click ABR) thresholds and auditory steady-state response (ASSR) thresholds among 55 infants and young children, 2 months to 35 months of age referred to the Ear Unit for electrophysiologic hearing assessment. Results: Click ABR showed strong positive correlation to all frequencies and averages of ASSR. Highest correlation was noted with the average of 1-4 kHz ASSR results with Pearson r = 0.89 (Spearman r=0.80), the average of 2-4 kHz had strong positive correlation r = 0.88 (0.79). Correlation was consistently strong through all ASSR frequencies (0.5 kHz at r=0.86 (0.74), 1 kHz at r=0.88 (0.78), 2 kHz at r=0. 87 (0.79), 4 kHz at r=0.85 (0.76)). Average differences of click ABR and ASSR thresholds were 8.2±12.9dB at 0.5 kHz, 8.6±12.6dB at 1 kHz, 5.3±11.8dB at 2 kHz and 7.8±13.4dB at 4 kHz. Among patients with no demonstrable waveforms by click ABR with maximal click stimulus, a large percentage presented with ASSR thresholds. Of these, 80.5% (33 of 41) had measurable results at 0.5 kHz with an average of 107.3±11.1dB, 85.4% (35 of 41) at 1 kHz with an average of 110.5±11.8dB, 73.2% (30 of 41) at 2 kHz with an average of 111.2±11.1dB and 63.4% (26 of 41) at 4 kHz with average of 112.2±8.21dB. Auditory steady-state response results were comparable to auditory brainstem response results in normal to severe hearing loss and provided additional information necessary for complete audiologic assessment especially among patients with severe to profound hearing loss wherein click ABR showed no responses. Up to 85.4% of patients that would have been noted to have no waveforms by click ABR still demonstrated measurable thresholds by ASSR. Conclusion: Our study suggests that ASSR may be the best available tool for assessing children with severe to profound hearing loss, and is a comparably effective tool in overall hearing assessment for patients requiring electrophysiological testing. The advantages of ASSR over click ABR include: 1) detection of frequency-specific thresholds and; 2) the detection of hearing loss thresholds beyond the limits of click ABR.

A 21 year old Filipino male presented with swelling of the second right hand digit unresponsive to antibiotics. Amputation revealed chronic inflammation and negative cultures. He developed sterile conjunctivitis and a generalized eruption of asymptomatic red papules and nodules. First skin biopsy revealed a diffuse infiltrate of epithelioid and foamy histiocytes, diagnosed as "juvenile xanthogranuloma." The second biopsy revealed large histiocytes with a "ground-glass" eosinophilic cytoplasm, multinucleated giant cells, and mixed cell infiltrate. Immunohistochemistry showed histiocytes staining with (+)S100 and (+)CD68, and (-)CD1a. Final diagnosis was "multicentric reticulohistocytosis." Despite treatment with oral prednisone, methotrexate and alendronate, lesions were progressive.
CONCLUSION: This fascinating case manifests with overlapping features of both juvenile xanthogranuloma and multicentric reticulohistiocytosis, and lead the authors to suggest considering the spectrum of diseases called the non-Langerhans cells histiocytosis when presented with a generalized nodular eruption.

Human ; Male ; Young Adult ; Antigens, Cd1 ; Biopsy ; Conjunctivitis ; Exanthema ; Giant Cells ; Histiocytes ; Histiocytosis, Non-langerhans-cell ; Inflammation ; Methotrexate

BACKGROUND: The long term complications of diabetes lead to diminished quality of life. As a means to avoid these complicationss, insulin therapy had evolved into a specialized regimen to achieve physiologic control of blood glucose, the so-called Multiple Daily Insulin (MDI) regimen which is a relatively new technique in the Philippines.
OBJECTIVE: To study the efficacy of Multiple Daily Insulin regimen in controlling plasma glucose and to evaluate patient's satisfaction with MDI regimen among Filipino type 2 diabetics who have been on MDI for at least 2 months.
RESEARCH DESIGN AND METHODS: Filipino patients (n=107) with uncontrolled diabetes were included in the study protocol. The majority were male, more than 60 years old and had been diabetic for more than 6 years. The mean body mass index was 27 kg/m2 and the mean baseline HbA1c was 9.2% + 2.65. This prospective cohort study was conducted between January 2006 to September 2008 at Cebu Doctors University Hospital Out-Patient Department. Efficacy was evaluated by HbA1c and treatment satisfaction was assessed with the Diabetes Treatment Satisfaction Questionnaire (DTSQ). Enrolled patients were followed up at week 12, 24 and 48.                                                                                                      
RESULTS: Mean HbA1c fell by 2.43 + 2.68 at week 12, 2.03 + 2.35 at week 24 and 1.73 + 2.23 at week 48 showing a statistically significant decrease of HbA1c among the 3 groups was not statistically significant (p = 0.52). The proportion of patients achieving an HbA1c of < 7% at week 12, 24, and 48 were 42%, 52% and 42%, respectively. Treatment satisfaction improved significantly (p = 0.000) with MDI regimen using the DTSQ at the end of the study.        
CONCLUSION: Multiple Daily Insulin Regimen is an effective treatment modality based on a significant lowering of HbA1c among the study population with uncontrolled type 2 diabetes mellitus. Patients were satisfied with the ragimen based on the positive treatment satisfaction. This, however, is contrary to popular belief that patients' quality of life is affected by insulin administration. Keywords: Multiple Daily Insulin Injection, Type Diabetes Mellitus, Treatment Satisfaction.

Human ; Male ; Female ; Aged ; Middle Aged ; Adult ; Blood Glucose ; Diabetes Mellitus, Type 2 ; Insulin ; Insulin Infusion Systems ; Outpatients ; Philippines ; Physician-patient Relations ; Prospective Studies ; Quality Of Life ; Treatment Outcome

INTRODUCTION: Interdigital pilonidal sinus is an acquired condition secondary to penetration of hair fragments into the skin of the web spaces of the hands commonly observed in hairdressers, and occasionally, among pet groomers. Local literature reports or guidelines to ensure practice of protective measures for this population of workers are currently lacking. CASE SUMMARY: A 24-year old pet groomer consulted due to occasional white hair strands emerging from two openings in the third interdigital space of his dominant hand. Histopathologic examination of the sinus tract showed an acanthotic, hyperplastic epidermis with scale crust, and nodular dermal infiltrates composed of epithelioid histiocytes, plasma cells, lymphocytes, and eosinophils. Transepidermal extrusion of polarizable hair cortical material was also evident establishing the diagnosis of an interdigital pilonidal sinus. Sinusectomy and debridement with healing by secondary intention resulted in an optimal wound closure and full motion of the affected hand after one week and minimal scarring with no recurrence after seven months. CONCLUSION Surgical excision followed by proper wound care is essential to avoid recurrence. In conclusion, since interdigital pilonidal disease is a rare condition, awareness among physicians would lead to accurate diagnosis, optimal treatment, and proper patient education.
occupational diseases ; grooming ; extremities ; Pilonidal sinus

INTRODUCTIONRecommended by the National Advisory Council of the Disabled, the Ministry of Health of Singapore supported a nationwide study of inherited metabolic disorders (IMDs). When the 5-year project ended, investigations were provided as a diagnostic service. This paper documents our 13-year experience.

MATERIALS AND METHODSPatients with symptoms suggestive of an IMD were referred. Investigations on heparinised blood and/or urine included amino acid analysis using a Beckman 6300 Amino Acid Analyser, organic acids analysis using a Hewlett- Packard gas chromatography and mass spectrometry, mucopolysaccharides quantitative assay and high-resolution electrophoresis, sugars by thin-layer chromatography.

RESULTSOf the 3656 patients studied from 1992 to 2005, IMDs were found in 127 (77 males; 50 females; age range, 1 day to 56 years). Their ethnic distribution was: 55.1% Chinese, 19.7% Malays, 11.0% Indians, 11.0% other races and 3.2% unknown. IMD diagnosed comprised 41 (32.3%) organic acidurias, 34 (26.8%) amino acidaemias/acidurias, 14 (11.0%) urea cycle defects, 15 (11.8%) mucopolysaccharidoses, 6 (4.7%) carbohydrate disorders and 17 (13.4%) others. Twenty-three (18.1%) cases were diagnosed during the neonatal period and 36 (28.3%) after the age of 13.

CONCLUSIONPositive detection rate was 3.5% and 48 IMDs were found. Significant proportion of cases had late-onset IMDs. Early identification of IMDs permits timely management, genetic counselling and prenatal diagnosis.

Adolescent ; Adult ; Amino Acids ; metabolism ; Biomarkers ; blood ; cerebrospinal fluid ; urine ; Carbohydrates ; blood ; Child ; Child, Preschool ; Chromatography, Gas ; Female ; Follow-Up Studies ; Glycosaminoglycans ; metabolism ; Humans ; Infant ; Infant, Newborn ; Male ; Mass Spectrometry ; Metabolism, Inborn Errors ; epidemiology ; metabolism ; Middle Aged ; Prevalence ; Prognosis ; Retrospective Studies ; Singapore ; epidemiology ; Urea ; metabolism

INTRODUCTIONThis paper shows the importance and value of external proficiency testing programmes in monitoring and improving a laboratory's diagnostic skills. It reviews and documents the wide variety of inherited metabolic disorders (IMDs) encountered in the programmes organised by the Human Genetics Society of Australasia and the College of American Pathologists.

MATERIALS AND METHODSThe programmes used actual patient specimens to assess a laboratory's ability to provide diagnoses based on laboratory tests results and brief clinical information. Participating laboratory was also required to suggest additional test(s) to confirm diagnoses.

RESULTSThe results of diagnoses on 116 samples were reviewed. Altogether 49 IMDs were encountered, including 26 organic acidurias, 16 aminoacidurias, 3 urea cycle defects, 5 mucopolysaccharidoses, and 1 each of mucolipidosis and purine disorder. Our report for 21 of the 116 samples (18.1%) deviated from the actual diagnoses. Deviations from the final diagnoses were recorded along with the reasons for them. The main reasons for the deviations were: the lack of standards for recognising metabolites of pathognomonic significance, absence of characteristic metabolites in samples collected during treatment, the presence of misleading unusual metabolites, inadequate clinical information, and inability to perform additional tests due to insufficient specimens.

CONCLUSIONSThe programmes provided a wide variety of IMDs, some of which we have yet to encounter in our patients. They also enabled us to learn about the varied biochemical manifestations at different stages of disease and the identity of previously unidentified metabolites. They enhanced our knowledge and experience and improved our diagnostic skills.

Australia ; Humans ; Laboratories ; standards ; Metabolism, Inborn Errors ; diagnosis ; New Zealand ; Pathology, Clinical ; standards ; Professional Competence ; Program Evaluation ; Quality Assurance, Health Care ; Quality Control ; Specimen Handling ; standards

Background: The incidence of diabetes mellitus is ever increasing. Individuals with diabetes mellitus may have concurrent mental health disorders and are shown to have poorer disease outcomes. The objectives of this study were to determine the prevalence of depression, anxiety and stress (DAS) in diabetes patients aged 20 years or more in the primary care setting. Methods: This was a cross-sectional study involving the use of self-administered questionnaire conducted in eight primary care private and government clinics in Pulau Pinang and Melaka, Malaysia. The validated DASS-21 questionnaire was used as a screening tool for the symptoms of DAS. Prior permission was obtained from the patients and, clearance from ethical committee was obtained before the start of the study. Data analysis was done using SPSS statistical software. Results: A total of 320 patients with diabetes from eight centres were enrolled via convenience sampling. Sample size was calculated using the Kish’s formula. The prevalence of DAS among patients with diabetes from our study was 26.6%, 40% and 19.4%, respectively. Depression was found to be significantly associated with marital status and family history of DAS; anxiety was significantly associated with monthly household income, presence of co-morbidities and family history of DAS; and stress was significantly associated with occupation and family history of DAS. Conclusions: The prevalence of DAS was higher in patients with diabetes compared with the general community. We recommend to routinely screen all patients with diabetes using the DASS-21 questionnaire because it is easy to perform and inexpensive.

The survival rate and prognosis for neonates with airway obstruction is poor if not managed immediately after delivery. Ex utero intrapartum treatment (EXIT) is indicated for cases in which airway obstruction is anticipated. The procedure establishes the fetal airway prior to complete delivery while maintaining an intact uteroplacental circulation. Maintaining uteroplacental circulation, ensuring uterine relaxation, and temporizing placental detachment during the EXIT procedure are achieved by administering a higher dose of inhalation anesthetic and intravenous nitroglycerine. However, this can lead to maternal hypotension and compromised feto-placental perfusion, reduced fatal cardiac output and acidosis. It is therefore essential that these be managed using vasopressors and inotropes. This paper reports the first institutional experience with the EXIT procedure in the Philippines, presenting two cases of neonates with large cystic hygroma. One case was performed as an elective procedure, the other as emergency treatment.

Human ; Female ; Adult ; Acidosis ; Airway Obstruction ; Anesthetics, Inhalation ; Cardiac Output ; Emergency Treatment ; Fetus ; Hypotension ; Infant, Newborn ; Lymphangioma, Cystic ; Philippines ; Placental Circulation ; Pregnancy ; Prognosis ; Survival Rate

INTRODUCTION: In the past decades, the prevalence of childhood overweight and obesity has increased worldwide. Childhood obesity has been associated with wide range of serious health complications and increased risk of premature adult illnesses. Non-alcoholic Fatty Liver Disease (NAFLD) was of concern because of limited data among children. The study aims to determine the prevalence and demographic /clinical factors associated with NAFLD among overweight and obese children.

METHODOLOGY: The study was a cross-sectional study among overweight and obese participants aged 2-18 years old. A total of 96 subjects were included. Frequencies and percentages of clinical characteristics were determined. Chi-square, linear correlation and logistic regression analysis for different factors were performed.

RESULTS: Among the 92 subjects, 26 (28%) were overweight while 66 (72%) were obese. The M:F ratio was 1.8:1 and majority belonged to 6-10 years old (44%). As to socioeconomic class, the rity (59%) were from the low-income group. The overall prevalence of NAFLD among overweight and obese subjects was 29.3%. None of the clinical factors (age, gender, socioeconomic status, BMI, waist circumference, actual caloric intake, and dietary fat consumption) were significantly associated with NAFLD. Analysis of biochemical factors revealed that alanine aminotransferase, aspartate aminotransferase, serum triglycerides and total cholesterol were found to be associated with NAFLD. Among which AST and ALT were identified predictors of NAFLD.

CONCLUSION: There was high prevalence of NAFLD among overweight and obese children. Screening among the pediatric population may aid on early identification and prevent its progression. ALT, AST, serum triglycerides and total cholesterol were independently related wih NAFLD. AST and ALT were identified predictors of NAFLD.

Human ; Overweight ; Obesity ; Child Nutrition Disorders ; Philippines ; Cross-sectional Studies

This is a rare case of a mucosal melanoma, located in the urethra of a 59-year-old male. Malignant melanomas of the genitourinary tract are rare, representing <1% of malignancies in the genitourinary tract, and <0.1% of all melanomas. In the male genitourinary tract, the most affected sites are the glans penis and the distal urethra in the fossa navicularis. Urethral melanomas comprise 4% of all urethral cancers.
Urethra