INTRODUCTION

Diffuse cutaneous mastocytosis (DCM) is a rare and severe form of cutaneous mastocytosis which may present in the neonatal period; thus early recognition is essential. Symptoms of mastocytosis are exacerbated by mast cell degranulating agents more commonly from heat, friction, local trauma, drugs and food. This is a case of DCM presenting with bullous eruptions after immunization.

CASE REPORT

An 11-month-old boy presented with generalized erythematous to hyperpigmented macules and patches initially at birth, with progression to bullous eruptions immediately after immunization without any systemic symptoms. Biopsy revealed superficial and deep mixed cell infiltrates consisting of lymphocytes, histiocytes and numerous mast cells. Giemsa stain highlighted the metachromatic mast cell granules. Serum tryptase was elevated by 13 times (130 ug/L). The patient was prescribed oral antihistamines and topical steroids that offered good response. Avoidance of all potential triggers was instructed.

DISCUSSION

The extensive cutaneous involvement in DCM (generalized erythema, diffuse papules that develop pachyderma, darker skin, peau d’orange) are due to the diffuse infiltration of the dermis with mast cells, accompanied with an elevated serum tryptase.

Unique to this local case are exacerbations triggered by vaccination. There is literature to support evidence of vaccination reactions to pentavalent vaccine in children with DCM though the pathway associated with mast cell degranulation after immunization has not yet been specified.

It is advised that patients with DCM follow scheduled immunization guidelines with precautionary measures.

Human ; Male ; Infant: 1-23 Months ; Wounds And Injuries ; Research Report ; Pharmaceutical Preparations ; Mastocytosis, Cutaneous ; Hot Temperature

BACKGROUND

Child malnutrition is a prevailing global public health concern especially in low- and middle-income countries. Conditional cash transfer (CCT) programs were implemented to help address this problem.

OBJECTIVE

To determine the relationship between the nutritional status among 0-59 months old children and household enrollment in a Philippine CCT program, Pantawid Pamilyang Pilipino Program (4Ps).

METHODS

A cross-sectional study was employed to 392 children and mothers/primary caregivers in a rural municipality in Leyte. Stratified random sampling technique was used in selecting the participants. Anthropometric characteristics were measured for these 392 children and were classified as 4Ps and non-4Ps members. Chi-square test was used to determine the relationship between the variables of interest.

RESULTS

4Ps household beneficiaries had mothers/primary caregivers who were older and had fewer years of education. The 4Ps beneficiary households had more household members and had lower average monthly income compared to the non-beneficiaries. No significant differences were found between the 4Ps beneficiary and non-beneficiary households in terms of the household hunger scale, the mean age of the children, and the sex distribution of the children included in the study. Specific profile components were found to be correlated to the children’s nutritional status. The age of the children was significantly associated to their length/height-for-age (L/HFA) wherein stunting was noted to occur among children older than 12 months of age. Maternal education was significantly associated to the weight-for-age (WFA) of the children. Children who were underweight had mothers/primary caregivers with fewer years of education. No significant correlation was found between the child’s sex, age of the mother/primary caregiver, household size, average monthly household income, and household hunger scale and the children’s nutritional status Lastly, there was no significant correlation between 4Ps household enrollment and the WFA and L/HFA status of the children. 4Ps household enrollment was, however, significantly correlated to the weight-for-length/height (WFL/H) or wasting status of the children.

CONCLUSION

The 4Ps program has the potential to enhance the nutritional outcomes of children hence the need to maximize its gains. In addition, the relationship of different sociodemographic variables with the children’s nutritional status reflects the complexity and multidimensionality of childhood malnutrition, implying the need for a holistic and multistakeholder approach in addressing the problem.

Human ; Infant Newborn: First 28 Days After Birth ; Infant: 1-23 Months ; Child Preschool: 2-5 Yrs Old ; Child Nutrition Disorders ; Nutritional Status ; Philippines

BACKGROUND

Stunting remains a major public health issue in Indonesia, particularly in regions like Pandeglang Regency, where prevalence rates are high. Understanding the contributing factors is essential for targeted interventions.

OBJECTIVE

This study aimed to analyze factors associated with stunting among toddlers using a mixed-methods approach.

METHODS

A cross-sectional mixed-methods study was conducted in two health centers in Banten Province from December 2021 to January 2022. Quantitative data were collected using a structured questionnaire and analyzed using chi-square tests (pRESULTS

There is a relationship between maternal knowledge, history of exclusive breastfeeding, history of supplementary feeding, support from health workers, and socio-economic status with the prevalence of stunting. However, the pattern of exclusive breastfeeding and seeking health services in the working areas of Bangkonol Health Center and Kaduhejo Health Center, Pandeglang Regency, is not good enough and needs to be improved. Health workers need to educate mothers with toddlers about patterns of providing complementary feeding, food preparation, and storage, and basic health practices to prevent stunting.

RESULTS

There is a relationship between maternal knowledge, history of exclusive breastfeeding, history of supplementary feeding, support from health workers, and socio-economic status with the prevalence of stunting. However, the pattern of exclusive breastfeeding and seeking health services in the working areas of Bangkonol Health Center and Kaduhejo Health Center, Pandeglang Regency, is not good enough and needs to be improved. Health workers need to educate mothers with toddlers about patterns of providing complementary feeding, food preparation, and storage, and basic health practices to prevent stunting.

Human ; Infant: 1-23 Months ; Child Preschool: 2-5 Yrs Old ; Public Health ; Prevalence ; Methods ; Indonesia ; Health ; Growth Disorders ; Comprehension

BACKGROUND

Six-pyruvoyl-tetrahydrobiopterin synthase (6-PTPS) deficiency is an inherited metabolic disorder which results in tetrahydrobiopterin (BH4) deficiency causing hyperphenylalaninemia.

OBJECTIVE

This study aimed to describe the clinical, biochemical, and radiologic profiles, and neurologic and developmental outcomes of patients diagnosed with 6-pyruvoyl tetrahydrobiopterin (PTPS) deficiency through newborn screening and confirmed by BH4 loading test, pterin analysis, and gene sequencing who were following-up with the metabolic team.

METHODS

The research was a single-center descriptive case series study design that was done at the Philippine General Hospital, a tertiary government hospital. The clinical, biochemical, radiologic profiles and neurodevelopmental evaluation of each patient were described.

RESULTS

Nine patients from 1 year 2 months to 14 years 5 months of age were enrolled in the study. Clinical manifestations before treatment were hypotonia, poor suck, and seizure. The most common clinical manifestation even after treatment initiation was seizure. The mean phenylalanine level on newborn screening was 990.68 umol/L, but after treatment was started, mean levels ranged from 75.69 to 385.09 umol/L. Two of the patients had focal atrophy of the posterior lobe on brain imaging. Pathogenic variants on molecular analysis were all missense, with two predominant variants, c.155A>G and c.58T>C. Eight of the nine patients had varying degrees of developmental delay or intellectual disability, while the remaining patient had signs of a learning disorder.

CONCLUSION

Newborn screening has played a crucial role in the early identification and management of patients with hyperphenylalaninemia due to 6-PTPS deficiency. Confirmation of diagnosis through determination of DHPR activity, urine pterins and/or molecular analysis is necessary for appropriate management. However, despite early initiation of treatment, neurodevelopmental findings of patients with 6-PTPS deficiency were still unsatisfactory.

Human ; Infant: 1-23 Months ; Child Preschool: 2-5 Yrs Old ; Child: 6-12 Yrs Old ; Adolescent: 13-18 Yrs Old ; Learning Disorders ; Brain ; Diagnosis

OBJECTIVE: This study described bronchiectasis profiles at the Philippine Children’s Medical Center (2021-2023). MATERIALS AND METHODS: A retrospective chart review at PCMC analyzed the demographic profile, imaging, management and outcome of patients with bronchiectasis enrolled in the Chronic Lung Disease Program from 2021-2023. Results: Twenty seven patients were included in this study. Most patients were diagnosed after the age of six and showed female predominance. The most common symptom at the time of diagnosis was chronic cough (81.5%) with pulmonary tuberculosis (51.9%) as the most common etiology. All patients were treated with cyclic azithromycin. Pseudomonas aeruginosa was the prevalent microorganism isolated in the sputum and tracheal aspirate samples of the patients (56.5%). Conclusion Patients with bronchiectasis were more commonly diagnosed in children past age of six with more prevalence in females. The most common symptom was chronic cough followed by fever, dyspnea and weight loss. Pulmonary tuberculosis and recurrent respiratory infections were noted to be the most common etiology with the left lower lobe most affected in HRCT. The most common phenotype seen was cystic, which is irreversible and a sign of progressive bronchiectasis, which may point to a late diagnosis. This emphasizes the need for physicians to have a high index of suspicion in patients with chronic or recurrent respiratory symptoms. All patients were treated with an oral macrolide with 25-31% of patients with decreased and/or absent symptoms. Pseudomonas aeruginosa was the prevalent microorganism isolated in the sputum and tracheal aspirate samples of the patients, which should be taken into account when treating for exacerbation.
Human ; Male,Female ; Infant newborn: First 28 days after birth ; Infant: 1-23 months ; Child Preschool: 2-5 yrs old ; Child: 6-12 yrs old ; Bronchiectasis ; Patients ; Tuberculosis ; Infections

INTRODUCTION

Neonatal sepsis is a significant cause of morbidity and mortality, particularly among preterm infants, and remains a pressing global health concern. Early-onset neonatal sepsis is particularly challenging to diagnose due to its nonspecific clinical presentation, necessitating effective and timely diagnostic tools to reduce adverse outcomes. Traditional methods, such as microbial cultures, are slow and often unavailable in resource-limited settings. This study aimed to develop a robust multivariable risk prediction model tailored to improve early detection of Early Onset Sepsis (EOS) among preterm infants in the Philippines.

METHODS

We conducted a retrospective analysis at a tertiary hospital in the Philippines using data from 1,354 preterm infants admitted between January 2019 and June 2024. Logistic regression models were employed, and predictors were selected through reverse stepwise elimination. Two scoring methods were developed: one based on beta coefficients divided by standard errors and another standardized to a total score of 100. The models were validated using Receiver Operator Characteristic curve analysis.

RESULTS

Version 1 of the scoring model demonstrated an Area Under the Curve (AUC) of 0.991, with a sensitivity of 90.91% and a specificity of 98.10%. Version 2 achieved an AUC of 0.999, with a sensitivity of 96.4% and a specificity of 92.44%.

CONCLUSIONS

The developed models provide a reliable, region specific tool for early detection of neonatal sepsis. Further validation across diverse populations and the integration of emerging diagnostic technologies, such as biomarkers and artificial intelligence, are warranted to enhance their applicability and accuracy.

Human ; Bacteria ; Infant: 1-23 Months ; Neonatal Sepsis ; Logistic Models ; Infant, Premature ; Philippines

Tufted angioma (TA) is a rare, benign, vascular neoplasm of the skin. The diagnosis of this condition is infrequent due to its rare occurrence. Only 158 cases have been described as of 2015. The treatment reported in the literature is very limited with no clear guidelines on its management. Currently, there are no reported cases in the Philippines of TA treated with aspirin. This is a case of a 1-year-old Filipino boy presenting with multiple dusky red papules and plaques on the left side of the cheek, pre- and postauricular areas, parieto-occipital areas, chest, and upper back. His lesions started at 2 months of age, noted to increase in size, number, and thickness over time. Dermoscopy revealed homogenous erythematous background with perifollicular lacunae separated by thin septa. Histopathology revealed dilated vessels in the papillary dermis with proliferation of endothelial cells in lobules, surrounded by dilated crescent-shaped vascular channels in the dermis. The patient was treated with low-dose aspirin (5 mg/kg/day) once a day for 1 month with improvement. After 4 months from treatment, no new lesions, no increase in size, nor symptoms were noted. Low-dose aspirin is an effective and safe option for monotherapy of TA in pediatric patients.

Human ; Male ; Infant: 1-23 Months ; Aspirin ; Tufted Angioma

The perioperative management of conjoined twins poses unique anatomic, physiologic, logistic, and even ethical challenges, necessitating a multidisciplinary team approach. We present a case of omphalopagus conjoined twins separated at the second month of life (39 2/7 weeks post-conceptual age). This paper highlights significant components in the anesthetic management of conjoined twins for separation surgery, in light of modern medicine and technological advancements.

Human ; Infant: 1-23 Months ; Twins, Conjoined ; Anesthesia

OBJECTIVE

To present a case of Langerhans cell histiocytosis mimicking bilateral otitis media and acute mastoiditis in a one-year-old girl, and to discuss the clinical presentation, diagnostic dilemma, management and prognosis of this disease.

METHODS

Design: Case Report

Setting: Tertiary Private Teaching Hospital

Patient: One

RESULTS

A one-year-old girl with a three-month history of bilateral otorrhea, post-auricular lymphadenopathies and dermatitits unresponsive to multiple courses of antibiotics, developed osseous and soft tissue destruction around the temporal and occipital bones. Her multiple progressive symptoms eventually led to the diagnosis of Langerhans cell histiocytosis.

CONCLUSION

Langerhans cell histiocytosis is a multifocal disease that can present with ear symptoms and can cause management dilemmas. It may mimic acute or chronic infections of the ear and should be suspected when extensive bone erosion is present.

Human ; Female ; Infant: 1-23 Months ; Histiocytosis, Langerhans-cell ; Otitis ; Mastoiditis

Human ; Male ; Female ; Infant: 1-23 Months ; Child Preschool: 2-5 Yrs Old ; Child: 6-12 Yrs Old ; Adolescent: 13-18 Yrs Old ; Urology ; Transitional Care ; Philippines ; Adult