A Case of Congenital Methemoglobinemia: Rare but Real.

Methemoglobin (MetHb) is a form of hemoglobin in which iron in Hb is in an oxidized form (ferric) instead of ferrous, making it difficult to bind with oxygen. Usually, MetHb is present in small quantities (<1%) in humans, but once MetHb increases beyond 3%, the condition is known as methemoglobinemia. It can be further classified into hereditary and acquired. Hereditary forms are a rare cause of hypoxia and cyanosis. Only a few cases have been reported worldwide. Here, we present a case of a 33-year-old female with congenital methemoglobinemia who remains relatively healthy in spite of her underlying condition. This case report focuses on knowledge sharing and practical aspects of managing patients with congenital methemoglobinemia.

Half-Dose Anticoagulation for Venous Thromboembolism and Phlegmasia Cerulea Dolens in a Patient With Recurrent Subdural Hematoma.

Anticoagulation for venous thromboembolism (VTE) in patients with recurrent subdural hematoma (SDH) is challenging. It becomes even more challenging when the patient develops phlegmasia cerulea dolens (PCD). We present a 66-year-old female with a recent history of recurrent SDH who received half-dose heparin therapy for VTE and PCD. The patient had improvement of dyspnea and resolution of PCD after two days of treatment. She was discharged with half-dose enoxaparin. At her one-month follow-up, there was no evidence of new SDH or progression of VTE. Half-dose anticoagulation therapy should be considered in patients with recurrent SDH when anticoagulation is inevitable.

A case report of recurrent pneumothoraces as a presentation of Birt Hogg Dube syndrome.

Birt Hogg Dube (BHD) syndrome, also called as Hornstein Knickenburg syndrome is a rare syndrome caused by germline mutation in the folliculin (FLCN) gene and transmitted via autosomal dominant pattern. Different phenotypes with different manifestations have been reported including, skin manifestations as fibrofolliculoma, lung manifestations as pulmonary cysts and spontaneous pneumothorax, renal manifestations as renal neoplasia and rarely colon polyps and colon cancer. We presented a case of 35-year-old female with Birt-Hogg-Dube Syndrome.

Sensitivity Study to Assess the Robustness of Primary Drying Process in Pharmaceutical Lyophilization.

The objective of this work is to apply a sensitivity study to assess the robustness of the primary drying step of pharmaceutical lyophilization with respect to deviations in process parameters. The sensitivity study can provide valuable information regarding the effect of process input parameters on the product quality that can aid in designing robust lyophilization processes. In this study, the output response is related to its inputs using Smolyak sparse grid generalized polynomial chaos method, and the sensitivity was calculated using elementary effects method. Sensitivity of chamber pressure and shelf temperature on product temperature of 2 sucrose-based and one mannitol-based formulation was studied, and the results were analyzed in terms of risk of adverse effects due to process deviations on the product quality. The study revealed that the sensitivity varies among formulations, and preliminary information regarding the possible impact of process deviations can be obtained from the process cycle diagram. The product temperature showed greater sensitivity toward the change in the shelf temperature than toward change in the chamber pressure for the greater part of the primary drying stage. An aggressive process-deviation scenario at the late stage of primary drying was also studied for different formulations, and the results were consistent with the sensitivity study.