Congenital diaphragmatic hernia in patient with 1p36 deletion.

Key Clinical Message: This case underscores the atypical presentation of late-onset congenital diaphragmatic hernia in a 9-old with 1p36 deletion syndrome. Recognition of respiratory distress and abdominal symptoms is crucial for intervention. Abstract: Congenital Diaphragmatic Hernia (CDH) is a condition characterized by the protrusion of abdominal contents into the thoracic cavity due to a defect in the diaphragm. While typically observed in the neonatal period, CDH can present in later life. This case report describes the presentation, diagnosis, and management of a nine-year-old boy with 1p36 deletion syndrome who presented with respiratory distress, abdominal pain, vomiting, and anorexia. The initial diagnosis was tension pneumothorax, and thus the patient underwent chest tube placement. However, a high-resolution CT scan revealed a left hemidiaphragmatic hernia, and the patient eventually underwent an emergency laparotomy due to acute-onset respiratory distress. Intraoperatively, a diagnosis of Bochdalek hernia with gastric perforation was made, and the CDH and gastric perforations were resolved successfully. This case highlights the importance of considering late-presenting CDH as a possible diagnosis in pediatric patients with similar symptoms and the radiological findings suggestive of tension pneumothorax. Early recognition and prompt surgical intervention can lead to successful management of such cases.

Effect of lactic acid spray on microbial and quality parameters of buffalo meat.

We evaluated the effect of lactic acid spray on micro-flora, instrumental color, shelf-life and sensory attributes of buffalo meat displayed under Modified Atmosphere Packaging. Buffalo calf carcasses (n = 12) were sliced into equal sagittal halves, n = 6 halves were randomly assigned to each of four treatments i.e. 2% LA, 4% LA, 6% LA and control. Afterwards, sirloin and tenderloin were vacuum packed and aged for 7 days. Later, steaks were packed in high-oxygen MAP. Microbial load, instrumental color, shelf-life and sensory attributes were evaluated at different days. Aerobic plate count of sprayed carcass and steaks was significantly lower than un-sprayed control. Similarly, though non-significant, redness and chroma value of sprayed carcass meat was found better than un-sprayed control. Lactic acid sprayed meat did not differ in terms of sensory attributes. It is concluded that spraying buffalo carcasses with 2-4% lactic acid after slaughter not only enhances microbial quality but it may also improve its instrumental color.

Association of Shah-Waardenburgh syndrome: a review of 6 cases.

UNLABELLED: Shah-Waardenburg syndrome (SWS) is a neurocristopathy and is characterized by Hirschsprung's disease (HD), deafness, and depigmentation of hairs, skin, and iris. OBJECTIVE: The aim of the article is to study the relative frequency of associations in 6 consecutive cases of SWS. METHODS: A review of 6 consecutive patients with SWS was performed to study the frequency of various components of the syndrome. RESULTS: Six patients had features of SWS. All patients had HD; of these, 3 had rectosigmoid HD, whereas 3 had extended HD. All patients had white forelock of hairs with skin depigmentation. One patient had sensorineural deafness, whereas other babies were less than 1 year, and thus, full evaluation of hearing deficiency was not assessed. Three patients had blue eyes, whereas other babies had normal iris pigmentation. Skin depigmentation was noted in 5 of the 6 patients. Three babies were seriously malnourished and showed higher association of enterocolitis. CONCLUSION: Shah-Waardenburg syndrome is an uncommon association of HD. Depigmentation with a white forelock and skin manifestations are common, whereas blue iris, long segment disease, and enterocolitis are present in nearly half of the patients.