Factor VII Deficiency: A Rare Case Report

Factor VII deficiency is a rare congenital coagulopathy. Prolonged prothrombin time with normal partial thromboplastin time indicates factor VII deficiency. For the definitive diagnosis, the specific factor VII level should be investigated. We report a 20 day old, baby girl, born full-term who was admitted with the diagnosis of sepsis. Hematological tests revealed prolonged prothrombin time and a factor VII level of nine percent. After antibiotic therapy and fresh frozen plasma replacement, her clinical status improved but the prothrombin time continued to be prolonged. On the seventh day of discharge the baby died due to sudden intracranial hemorrhage.     

Tuberculosis of transverse testicular ectopic testis associated with persistent mullerian duct syndrome

A 10-month-old male infant presented with bilateral inguinal hernia and left un-descended testis. During right herniotomy, both gonads were found on same side with mullerian duct structures. On naked eye examination, both gonads were normal looking. Excision of mullerian duct remnant and fixation of ectopic testis was made. Histopathological examination revealed that gonads were testicles. Presence of multiple granulomas composed of Langhans cells and epithelioid cells in ectopic testicle suggested tuberculosis. Patient was kept on antituberculous therapy and was on regular follow-up without any complication.     

Lupus vulgaris with tubercular lymphadenitis and IgA nephropathy

A 14-year-old girl presented with a 10-year history of a large crusted plaque over the right thigh for 10 years and small reddish plaque over the left upper back for 3 months. On routine evaluation, she was found to have hematuria. Skin biopsy from the lesion was suggestive of skin tuberculosis (lupus vulgaris), and kidney biopsy showed features of IgA nephropathy (IgAN). Fine-needle aspiration from the inguinal lymph node was consistent with granulomatous disease. The patient has been on anti-tubercular treatment, and the hematuria has subsided.     

Lesional T-cell subset in post-kala-azar dermal leishmaniasis

BACKGROUND: The pathogenesis of post-kala-azar dermal leishmaniasis (PKDL) is ill understood. This study was carried out to find the percentage of T-helper and T-suppressor cells in lesional tissue and their probable role in the pathogenesis of PKDL. METHODS: An immunoperoxidase monoclonal antibody technique was used to characterize and quantify the subsets of T lymphocytes in the infiltrate in 25 patients with PKDL. RESULTS: The ratio of T-helper to T-suppressor cells was 0.87 in hypopigmented macules and 0.85 in nodule and/or plaque lesions of PKDL. CONCLUSIONS: In this study, there was a definite preponderance of T-suppressor cells over T-helper cells in both types of skin lesions of PKDL. Further studies should be undertaken on larger numbers of patients to compare T-cell subsets both in skin lesions and the circulation, in order to determine the pathogenesis of PKDL.     

Meconium peritonitis presenting as abdominal calcification: three cases with different pathology

Intra-abdominal calcification is uncommon in newborns and has several causes of which meconium peritonitis is the most frequent. Three neonates with intra-abdominal calcification as a complication of meconium peritonitis are presented. The types of meconium peritonitis were cystic, meconium pseudocyst and meconium ascites. Two required surgical intervention. Meconium peritonitis should be considered in newborns with intra-abdominal calcification.     

Association of Nutrition Education and Its Interaction with Lifestyle Factors on Kidney Function Parameters and Cardiovascular Risk Factors among Chronic Kidney Disease Patients in Taiwan

We evaluated the interactive effects of nutrition education (NE) and lifestyle factors on kidney function parameters and cardiovascular risk factors among chronic kidney disease (CKD) patients. This cross-sectional cohort study recruited 2176 CKD stages 3–5 patients aged > 20 years from Integrated Chronic Kidney Disease Care Network, Shuang Ho Hospital, Taiwan between December 2008 and April 2019. The multivariable regression analysis was performed to investigate the interactive effects of NE with lifestyle factors on kidney function parameters and cardiovascular risk factors. Relative excess risk due to interaction (RERI) and attributable proportion (AP) were applied to assess additive interaction. Patients who were smoking or physically inactive but received NE had better estimated glomerular filtration rate (eGFR) (β: 3.83, 95% CI: 1.17–6.49 or β: 3.67, 95% CI: 2.04–5.29) compared to those without NE. Patients with smoking and NE significantly reduced risks for having high glycated hemoglobin A_1c (HbA_1c) by 47%, high low-density lipoprotein cholesterol (LDL-C) by 38%, and high corrected calcium (C-Ca) by 50% compared to those without NE. Moreover, NE and smoking or inactive physical activity exhibited an excess risk of high C-Ca (RERI: 0.47, 95% CI: 0.09–0.85 for smoking or RERI: 0.46, 95% CI: 0.01–0.90 and AP: 0.51, 95% CI: 0.03–0.99 for physical activity). Our study suggests that CKD patients who were enrolled in the NE program had better kidney function. Thus, NE could be associated with slowing kidney function decline and improving cardiovascular risk factors.