L-arginine as a medication

Background. L-arginine takes part in the ornithine cycle, in which ammonia is neutralized with urea formation. Accordingly, in the absence of L-arginine, toxic ammonia accumulates in the organism. Objective. To describe the use of L-arginine as a medication. Materials and methods. Analysis of literature sources on this topic. Results and discussion. Nitric oxide (NO) is a universal mediator in the regulation of cellular functions and intercellular communication. Deficiency of this substance is a key element of endothelial dysfunction. In the human body, NO is produced from L-arginine and performs an extremely important function of vasodilation due to the relaxation of vascular smooth muscle cells. Other physiological functions of NO include the transmission of neural signals in the central and peripheral nervous system, nervous activity, histamine secretion by mast cells, intestinal peristalsis, erection, killer action against bacteria and cancer cells. Arginine deficiency occurs in hypertension, coronary heart disease, obliterating disease of peripherl arteries, primary pulmonary hypertension, obstetric and perinatal pathological conditions. Potential ways to eliminate NO deficiency include the administration of its precursor – L-arginine (Tivortin, “Yuria-Pharm”) or its donors (molsidomine, nitrates). Tivortin has antioxidant and detoxifying effects, reduces endothelial dysfunction, activates urea synthesis, promotes glucose utilization, increases blood insulin. Conclusions. 1. NO deficiency is a key element of endothelial dysfunction. 2. Physiological functions of NO are vasodilation, transmission of neural signals, intestinal motility, etc. 3. In the human body NO is formed from L-arginine. 4. Arginine deficiency occurs in hypertension, coronary heart disease, obliterating disease of the peripheral arteries, primary pulmonary hypertension, obstetric and perinatal pathological conditions. 5. L-arginine (Tivortin) prescription is one of the ways to eliminate NO deficiency. 6. Tivortin has antioxidant and detoxifying effects, reduces endothelial dysfunction, and promotes glucose utilization.

Diagnosis and management of congenital umbilical arteriovenous malformation

An 8-day-old neonate was presented with severe respiratory distress and diagnosed as primary pulmonary hypertension of the newborn on functional echocardiogram. Evaluation showed bounding pulse, enlarged umbilical cord, and bruit over the periumbilical region. Transthoracic echocardiography and CT angiogram showed a large fistulous communication between the umbilical vein and artery suggestive of congenital umbilical arteriovenous malformation leading to high-output cardiac failure and pulmonary artery hypertension. The patient was stabilised with medications and ventilation. Transcatheter closure of communication was done using coils, vascular plug, and KONAR-MFTM device. The patient improved from heart failure soon after the procedure and thriving normally at 6 months of follow-up.

Risk factors for 30-day readmission in adults hospitalized for pulmonary hypertension

Readmissions for pulmonary hypertension are poorly understood and understudied. We sought to determine national estimates and risk factors for 30-day readmission after pulmonary hypertension-related hospitalizations. We utilized the Healthcare Cost and Utilization Project Nationwide Readmission Database, which has weighted estimates of roughly 35 million discharges in the US. Adult patients with primary International Classification of Disease, Ninth Revision, Clinical Modification diagnosis codes of 416.0 and 416.8 for primary and secondary pulmonary hypertension with an index admission between 2012 and 2014 and any readmission within 30 days of the index event were identified. Predictors of 30-day readmission were identified using multivariable logistic regression with adjustment for covariates. Results showed that the national estimate for Primary Pulmonary Hypertension vs Secondary Pulmonary Hypertension-related index events between 2012 and 2014 with 30-day readmission was 247 vs 2550 corresponding to a national readmission risk estimate of 17% vs 18.3%, respectively. The presence of fluid and electrolyte disorders, renal failure, and alcohol abuse were associated with increased risk of readmission in Primary Pulmonary Hypertension, while factors associated with Secondary Pulmonary Hypertension readmissions included anemia, congestive heart failure, lung disease, fluid and electrolyte disorders, renal failure, diabetes, and liver disease. The median cost of Primary Pulmonary Hypertension admissions and readmissions were $46,132 (IQR: $25,384–$85,647) and $41,604.50 (IQR: $22,481.50–$84,420.50), respectively. The median costs of Secondary Pulmonary Hypertension admissions and readmissions were $34,893 (IQR: $19,670–$66,143) and $36,279 (IQR: $19,059–$74,679), respectively. In conclusion, approximately 19% of Primary Pulmonary Hypertension and Secondary Pulmonary Hypertension hospitalizations result in 30-day readmission, with significant costs accrued during the index hospitalization and readmission. With evolving clinical terminology and diagnostic codes, future study will need to better clarify underlying factors associated with readmissions amongst pulmonary hypertension sub-types, and identify methods and procedures to minimize readmission risk.

Sudden Vision Loss in a Young Patient with Pulmonary Hypertension, A Rare and Unusual Finding

Loss of vision associated with pulmonary hypertension in the pediatric age group has been rarely reported in the literature and often goes unnoticed for a long period. Pulmonary hypertension may manifest as shortness of breath limiting the activity of the individual, palpitation, headaches, easy fatiguability, dizziness been the most common symptoms encountered. However, primary pulmonary hypertension would not present itself instead secondary to other diseases like congenital heart disease, lung disease, connective tissue disorders or genetic diseases. Our case demonstrated a 17-year old individual with a primary complaint of progressive loss of vision in the left eye leading to complete vision loss. The patient experienced dyspnea two years back which exacerbated affecting his normal routine activity. His Echocardiography revealed severe pulmonary hypertension and was started on appropriate medications. His vision worsened and slit-lamp examination revealed multiple hemorrhages. Further investigations with FFA and OCT revealed significant hypoperfusion and significant elevation of the retina of the left eye. Anti-VEGF injections were given in an attempt to restore vision, but it resulted in no further improvement. Our case emphasizes the importance of prompt ophthalmologic examination in Pulmonary Hypertension.