Dynamic Self-Determination of Self-Care and Positive Deviance Model for Stunting Prevention in Indonesia

ABSTRACT Objective: To describe the dynamic self-determination of self-care (DSDoSC) and positive deviance (PD) models in changing stunting prevention behavior. Material and Methods: This research is a quasi-experimental study with a sample of 90 mothers taken by purposive sampling. Thirty mothers were given the DSDoSC intervention, 30 were given the PD intervention, and another 30 were in the control group. This research was conducted in July - October 2019. The variables studied were feeding behavior, nurturing behavior, personal hygiene behavior, environmental cleanliness and air sanitation, and behavior seeking health services. To analyze the difference in mother behaviour before and after test, we used Paired t-test. Analysis of Variance (MANOVA) was used to analyze the difference of mother behaviour among groups. The level of significance was p<0.05. Results: The PWD group showed that eating behavior, parenting behavior, personal hygiene behavior, environmental hygiene and water sanitation, and behavior seeking health services had significant numbers. In the DSDoSC group, eating behavior, parenting behavior, environmental hygiene, water sanitation and health service-seeking behavior were significantly (p<0.05). The results of the Manova test showed that there was an effect of PD and DSDoSC on stunting prevention behavior. Conclusion: Self-dynamic for self-care model and the positive deviance model both can change a mother's behavior for the better in feeding, parenting, environmental hygiene, and water sanitation, seeking health services, but not changing behavior about personal hygiene behavior.

De Sanctis-Cacchione Syndrome in a female infant - Case report / Sindrome de De Sanctis-Cacchione em lactente do sexo feminino - Relato de caso

The De Sanctis-Cacchione Syndrome is the rarest and most severe kind of xeroderma pigmentosum, characterized by microcephaly, hypogonadism, neurological disorders, mental and growth retardation, with very few cases published. The clinical findings compatible with De Sanctis-Cacchione Syndrome and the therapeutic approach used to treat a one year and nine months old child, with previous diagnosis of xeroderma pigmentosum, are reported.

A síndrome de de Sanctis-Cacchione é a forma mais rara e grave do xeroderma pigmentoso e é caracterizada por microcefalia, hipogonadismo, alterações neurológicas e retardo mental e de crescimento, com poucos casos publicados. Relatam-se os achados clínicos compatíveis com essa síndrome e a terapêutica instituída em uma lactente de um ano e nove meses, com diagnóstico prévio de xeroderma pigmentoso.

Seckel syndrome with chromosomal 18 deletion.

Four case records of patients with Seckel Syndrome (SS) were retrieved. Typical of bird headed dwarfism was seen in all. Chromosome 18 deletion was seen in one child with SS. MRI abnormalities were detected in 3 patients. Cytogenetic studies and neuroimaging is likely to provide important diagnostic and prognostic information.

Metatropic dysplasia in children.

Metatropic dysplasia is a rare genetic condition characterized by progressive dwarfism. Metatropic dysplasia is defined as a short limb skeletal dysplasia characterized by dumbell like configuration of long bones, a narrow but normal length of thorax and occasionally a coccygeal appendage similar to a tail. Children born with this condition show different signs and symptoms throughout the childhood. An eight years old boy was admitted in the Paediatric ward of Bangbandhu Sheikh Mujib Medical University (BSMMU), Dhaka with the complain of swelling in the back for 7 1/2 years. On examination the boy had features suggestive of Metatropic dysplasia. Radiological findings included exaggerated curvature of the spine which was different at the different levels giving rise to "S" shaped deformity, pectus carinatum and flattening of the vertebral bodies which were variable at different levels. From the history, clinical examination and radiological findings the boy was diagnosed as a case of metatropic dysplasia.

Pulmonary hypoplasia in Jarcho-Levin syndrome

Jarcho-Levin syndrome, also known as spondylothoracic dysplasia and characterized by short trunk dwarfism, "crab-like" rib cage, with ribs and vertebral defects; it is not uncommon in Puerto Ricans. Many patients die in early infancy due to respiratory compromise associated to lung restriction and the reported cases emphasize mostly the skeletal malformations associated to the syndrome. We report the autopsy findings in a newborn with isolated Jarcho-Levin syndrome emphasizing pulmonary pathology. He was a pre-term male who died of respiratory failure at three hours old and, autopsy findings confirmed the clinical diagnosis. Internal examination showed hypoplastic lungs with normal lobation. The histological structure appeared normal and relatively mature; the diaphragm showed eventration and unilateral absence of musculature. This case shows the worst spectum of the Jarcho-Levin syndrome: pulmonary hypoplasia not compatible with extrauterine life. Since thoracic restriction is present during the fetal period, the degree of pulmonary hypoplasia probably defines survival beyond the neonatal period.