Gaucher Disease: A First Reported Adult Case in Indonesia.

A 44-year-old female presented with a distended abdomen and fatigue. On physical examination, prominent splenomegaly was found. The laboratory investigations revealed pancytopenia and decreased albumin-globulin ratio. The abdominal ultrasonography revealed splenomegaly, cholelithiasis, and cystitis, and the bone survey showed osteopenia. Differential diagnoses included leukemia, multiple myeloma, and myelofibrosis therefore bone marrow puncture was performed. However, histopathologic examination found Gaucher-like cells in the bone marrow aspiration. The finding of CD68 positivity in Gaucher-like cells by using the immunohistochemistry staining supporting Gaucher disease. To confirm the diagnosis, an examination of glucocerebroside substrate from the patient's blood plasma was performed. Glucosylsphingosine, a deacylated form of glucosylceramide, was markedly elevated. Therefore, the diagnosis of Gaucher disease was confirmed. This is the first reported adult Gaucher case diagnosed in Indonesia.

Effectiveness of a comprehensive integrated module using interactive lectures and workshops in understanding and knowledge retention about infant feeding practice in fifth year medical students: a quasi-experimental study.

BACKGROUND: Sixty percent of the 10.9 million under-5 deaths every year are related to malnutrition. More than two thirds of malnutrition is caused by inappropriate infant feeding practice. Only 35 % of mothers worldwide provide 4 months of exclusive breast-feeding, while complementary feeding is often untimely, nutritionally inadequate, hygienically poor, and improperly delivered. The existing pediatric nutrition module in our institution does not include proper delivery of food that involves oral-motor skills and feeding behavior. To scale up the knowledge and skill of medical students regarding evidence-based infant feeding practice, we designed a new module composed of comprehensive and integrated lectures with additional multidisciplinary lectures on oral-motor skill development and feeding behavior. METHODS: A quasi-experimental study was conducted to evaluate the efficacy of the new module compared to the previous module. Fifth year medical students of Universitas Indonesia were divided into intervention and control groups. The control group received lectures and a paper-based workshop. The intervention group received comprehensive and integrated interactive lectures with additional multidisciplinary lectures on oral-motor skill development and behavioral approaches to feeding problems. A hands-on workshop using real cases shown on recorded video and role-play sessions was also presented to the intervention group. A pre-/post-test, 3-month retention test, and Observed Structured Clinical Examination (OSCE) were performed to evaluate understanding, knowledge retention, and counseling skills. RESULTS: A linear mixed effect model with a random intercept analysis for pre-test, post-test, and retention test scores showed significant higher result for intervention group compared to control group (p < 0.001). Comprehensive knowledge and counselling skills were better in the intervention group than in the control group as shown by the OSCE score (68.6 vs 59.3, p < 0.001). CONCLUSIONS: Our comprehensive integrated infant feeding practice module, which incorporates multidisciplinary learning processes and an interactive hands-on workshop with a role-play session yields better knowledge understanding and counseling skills compared with the existing module. Comprehensive knowledge and good counseling skills of medical students as future doctors are a pre-requisite to provide effective education to parents to support successful infant feeding practices.

Role of antioxidants in reducing oxidative stress and seizure frequency in drug-resistant epileptic patients.

Drug-resistant epilepsy presents significant challenges in treating epileptic patients, leading to recurrent seizures and necessitating the use of polypharmacy with anti-epileptic drugs. Both of these conditions contribute to increased oxidative stress, which is detrimental to the brain. The aim of this study was to determine the role of vitamins C and E in reducing oxidative stress and seizure frequency in drug-resistant epileptic patients. This was a double-blinded, randomized clinical trial with a placebo, parallel design, and block randomization. The subjects were drug-resistant epileptic patients aged 1-18 years who received routine treatment. Randomization was performed on 100 patients who were divided into the treatment or placebo groups. The patients received a combination of vitamin C (100 mg/day) and vitamin E (200 IU/day for those <5 years or 400 IU/day for those ≥5 years) or a placebo for eight weeks. Malondialdehyde (MDA) levels and seizure frequency were measured prior to and after the intervention. A total of 42 and 46 patients were followed till the end of the study in the intervention and placebo groups, respectively. Our data indicated that the MDA levels prior to treatment were not significantly different between the treatment and placebo groups (0.901 vs 0.890 mmol/mL, p=0.920) and were significantly reduced after the treatment in both the treatment group (p<0.001) and placebo group (p=0.028). The changes in MDA levels (between post- and pre-treatment) were also not significantly different between the two groups (p=0.181). Our per-protocol analysis indicated that the reduction in seizure frequency was significantly higher in the treatment group compared to the placebo group (95% vs 35%, p<0.001), with 92% and 60% relative and absolute risk reduction, respectively. The intention-to-treat analysis also indicated that the reduction in seizure frequency was significantly higher in the intervention group than in the control group (80% vs 32%, p<0.001), with relative and absolute risk reduction of 70% and 48%, respectively. There was no significant relationship between changes in MDA levels and seizure frequency in either group. In conclusion, vitamins C and E could reduce seizure frequency and, therefore, could be considered as adjuvant therapy in drug-resistant epileptic patients.

Invasive pneumococcal disease among hospitalized children aged 28 days to 60 months in Jarkarta.

Streptococcus pneumoniae is a leading cause of morbidity and mortality among children worldwide. Prevention of invasive pneumococcal disease (IPD) with a pneumococcal conjugate vaccine (PCV) is an effective approach to reduce the burden of pneumococcal disease. Nationwide epidemiological data is required prior to considering universal pneumococcal immunization for Indonesia. This preliminary study aimed to quantify the burden of IPD among hospitalized children at Cipto Mangunkusumo Hospital and Fatmawati Hospital, Jakarta. We studied 205 subjects aged 28 days to 60 months who were admitted with the diagnosis of pneumonia, meningitis, sepsis, and suspected occult bacteremia. Streptococcus pneumoniae was isolated from 1 of 205 blood specimens, giving an IPD proportion of 0.5%. The IPD case in this study was a 3-month-old baby with meningitis and bilateral lobar pneumonia. The Quellung test demonstrated serotype 7F. The isolate was susceptible to amoxicillin and Cotrimoxazole. Incidence of IPD could not be calculated due to low number of cases; this underscores the importance of surveillance of pneumococcal disease in Indonesia.