The sustainable use of diverse plants accustomed by different ethnic groups in Sibi District, Balochistan, Pakistan.

The present study was conducted to analyze the utilization of medicinal plants (traditional as well as cultivated) and there recipes accustomed by different ethnic groups of Sibi District (SD), Balochistan, Pakistan. The study was carried out between 2018 and 2021 by using semi-structured and open-ended questionnaire.. The randomly selected methods applied for this study were mainly based on household surveys walk through and interview with indigenous communityage 40 to 80, a total of 75 plants, belonging to 63 genera and distributed among 33 plant families were recorded. The dominant Plant families were the Fabaceae (12%) of all studied taxa, followed by the Amaranthaceae (7%), Asteraceae (6%), Cucurbitaceae, Solanaceae, Poaceae (4% each), Rhamnaceae and Zygophyllaceae (3%). Thirty traditional Food Recipes (TFR) and Traditional Medicinal Recipes (TMR) were novel being first time reported from SD., which are utilized by the local communities in their daily routine. These ethnic TFR and TMR have a tremendous role in preservation and sustainable use of traditional food habits and culture. It was also documented that along with cultivated, the wild edible and medicinal plant preparations play a significant role in in the economic potential and primary health care system of the local communities. The study recommends the specific measures, such as small industries, improved export means, tourism and educational activities, to protect the traditional knowledge and biocultural heritage of the region before its erosion.

Quantifying medical manifestations in Hurler syndrome with the infant physical symptom score: associations with long-term physical and adaptive outcomes.

BACKGROUND: A physical symptom score (PSS) for the mucopolysaccharidosis (MPS) disorders has been developed to quantitate the somatic burden of disease across multiple organ systems. Studies have demonstrated the sensitivity and its relationship to age, IQ and adaptive functioning of the PSS in older children. With the onset of newborn screening, there is an increased need to characterize the somatic symptoms in the earliest stages of life, especially for young children under 36 months of age. Consequently, a new scale, Infant Physical Symptom Score (IPSS), was developed to score physical symptoms in infants and toddlers. OBJECTIVE: Part I. To create a measure to quantify somatic burden in patients with MPS disorders under 36 months of age. The IPSS assess outcomes and changes in somatic disease in individuals with MPS disorders diagnosed very early in life. Part II. To determine the relationship between IPSS and other measures to evaluate its validity and utility, a) we evaluated the relationship between the IPSS and PSS in the same patients with MPS I over time to determine if the two scales are measuring the same concepts, and b) we evaluated the association between IPSS and a functional adaptive measure over time with a focus on the age at first treatment (under 36 months) to determine if the IPSS has predictive value. METHODS: Part I. The Infant Physical Symptom Score (IPSS) for the infant population in MPS disorders was established using data from 39 patients enrolled in the Lysosomal Disease Network longitudinal MPS I study (U54NS065768). All of these patients had Hurler syndrome (MPS IH) and underwent hematopoietic stem cell transplant (HSCT) at the University of Minnesota. Items for the IPSS were selected by reviewing CRFs prepared for the MPS I longitudinal study and examining medical records of these patients prior to HSCT based on the knowledge gained from the development of the PSS. Part II. Of those 39 patients, a subset of 19 were all seen 9 to 12 years post HSCT. Having retrospectively calculated their IPSS prior to HSCT, we categorized them by age at HSCT, and examined their most recent PSS along with Composite and Daily Living Skills scores on the Vineland Adaptive Behavior Scales - Second Edition (VABS-II). RESULTS AND CONCLUSION: The total score on the IPSS collected prior to transplant differed by patient's age at transplant, as expected in this progressive condition. Those transplanted at ≤12 months of age had a mean score of 7.4, which was significantly lower, suggesting less somatic disease burden, compared to those transplanted at >12 to ≤24 months (mean 11.8) and > 24 to ≤36 months (mean 13.6). Higher IPSS reflects more evidence of somatic disease burden and lower IPSS reflects less evidence of disease burden. Nine to 12 years later, the severity level as measured by the PSS was comparable to severity on the IPSS suggesting that the two scales are measuring similar concepts. Retrospectively calculated pre-transplant IPSS were negatively associated with higher VABS-II Composite scores 9-12 years later (p value-0.015) and to a lesser extent Daily Living Skills scores (p value-0.081). We conclude that the IPSS appears to be a useful approach to quantifying the somatic disease burden of MPS IH patients under 36 months of age.