Efficacy of 0.5% timolol maleate gel in the management of infantile hemangiomas.

Objective: This study aimed to evaluate the efficacy and safety of 0.5% timolol maleate gel, reported as an efficacious option for management of infantile hemangiomas (IH) in children. Methods: A retrospective study was conducted among patients diagnosed with IH from January 2019 to December 2021. All patients were treated 0.5% timolol gel. Data parameters, including photographs, at baseline and the final or most recent follow-up visit, were reviewed. Outcomes based on photographic assessment were categorized as excellent, good, fair or poor. Results: Sixty-four children with 76 lesions were enrolled. Median age was eight months (two months to 36 months) with most lesions (75.0%) presenting during the first year of life. Female preponderance (84.4%) was seen and the cervicofacial region was most commonly involved (52.6%). The majority of lesions (54, 84.4%) were solitary and most were treatment naïve (n=61, 80.3%). Excellent, good, fair, and poor responses were seen in 24 (31.5%), 39 (51.3%), 6 (7.9%), and 7 (9.2%) lesions. No complications were seen and no statistically significant difference was observed with respect to gender, age group, region involved and treatment naïve versus previously treated patients. Conclusion: Timolol maleate 0.5% gel is an effective and safe treatment option for IH irrespective of location of lesion, age and history of prior treatment.

Kasabach-Merritt Syndrome: a case study of successful treatment with vincristine and propranolol.

Kasabach-Merritt syndrome is a rare condition, characterised by the presence of an enlarging vascular tumour associated with thrombocytopenia, microangiopathic haemolytic anaemia and consumptive coagulopathy. The syndrome manifests in infancy, with high morbidity and mortality rates. No standard guidelines have been established for the treatment of Kasabach-Merritt syndrome to date. To existing literature we add this report of a four-month-old female child with Kasabach-Merritt syndrome who was successfully treated with propranolol and vincristine. This drug combination helped reverse the severe thrombocytopenia as well as decrease in size of her haemangioma. Management of Kasabach-Merritt syndrome continues to be a challenge, with varying response to first line drugs. Early diagnosis and initiation of treatment in a closely monitored setting is essential to ensure good outcomes. Since this is a relatively rare condition and large studies are not feasible, documenting treatment experience for single cases or small series becomes even more important.

Ring forming transformations of ynamides via cycloaddition.

Ynamides are N-alkyne compounds bearing an electron withdrawing group at the nitrogen atom. They offer unique pathways for the construction of versatile building blocks owing to their exceptional balance between reactivity and stability. Recently several studies have been reported that explore and illustrate the synthetic potential of ynamides and ynamide-derived advanced intermediates in cycloadditions with different reaction partners to yield heterocyclic cycloadducts of synthetic and pharmaceutical value. Cycloaddition reactions of ynamides are the facile and preferable routes for the construction of structural motifs having striking importance in synthetic, medicinal chemistry, and advanced materials. In this systematic review, we highlighted the recently reported novel transformations and synthetic applications that involved the cycloaddition reaction of ynamides. The scope along with the limitations of the transformations are discussed in detail.

Assessment of outcomes after intralesional bleomycin sclerotherapy of lymphatic malformations in children.

OBJECTIVE: To evaluate the efficacy of bleomycin in the treatment of lymphatic malformations, and the concordance between photographic and radiological assessments of the outcome. METHODS: The retrospective study was conducted at the Vascular Anomalies Centre of Indus Hospital, Karachi, and comprised data of patients enrolled with diagnosis of macrocystic or mixed lymphatic malformations from January 2017 to November 2019. All patients had been treated with injection bleomycin 0.6-1mg/kg/session. Size and location of lesions, ultrasonographic findings, photographic documentation and post-procedure complications were reviewed. Photographic and radiographical assessment outcomes were categorised as excellent, good or poor, and compared for concordance. Data was analysed using Stata 14. Results: Of the 31 children, 22(68.8%) were boys. Mean age at presentation was 54.2±44 months (range: 2 months to 15.7 years). There were 32 lymphatic malformations; 29(90.6) macrocystic and 3(9.4%) mixed. Head and neck region was mostly involved 19(59.4%). Most lesions 23(71.9%) presented during the first year of life, and 29(90.6%) lesions were purely macrocystic. Excellent, good and poor response was seen in 16(50%), 15(46.9%) and 1(3.1%) lesions on photographic assessment, and 21(65.6%), 11(34.4%) and 0(0.0%) lesions on radiological assessment, respectively. Concordance in photographic and radiological outcomes was 22(69%). No complications were seen and no statistically significant difference was observed for photographic and radiographic assessment with respect to gender, malformation type, region involved, and number of sessions (p>0.05). CONCLUSIONS: Intralesional bleomycin sclerotherapy was found to be effective in the treatment of lymphatic malformations. Clinical observation was reliable in assessing progress on routine follow-up, with additional radiology done when management decisions needed to be reviewed.

One-Pot Multicomponent Synthesis and Bioevaluation of Tetrahydroquinoline Derivatives as Potential Antioxidants, α-Amylase Enzyme Inhibitors, Anti-Cancerous and Anti-Inflammatory Agents.

Mankind has always suffered from multiple diseases. Therefore, there has been a rigorous need in the field of medicinal chemistry for the design and discovery of new and potent molecular entities. In this work, thirteen tetrahydroquinoline derivatives were synthesized and evaluated biologically for their antioxidant, α-amylase enzyme inhibitory, anti-proliferative and anti-inflammatory activities. SF8 showed the lowest IC50 of 29.19 ± 0.25 µg/mL by scavenging DPPH free radicals. SF5 showed significant antioxidant activity in total antioxidant capacity (TAC) and total reducing power (TRP) assays. SF5 and SF9 showed the maximum inhibition of α-amylase enzyme i.e., 97.47% and 89.93%, respectively, at 200 µg/mL concentration. Five compounds were shortlisted to determine their anti-proliferative potential against Hep-2C cells. The study was conducted for 24, 48 and 72 h. SF8 showed significant results, having an IC50 value of 11.9 ± 1.04 µM at 72 h when compared with standard cisplatin (IC50 value of 14.6 ± 1.01 µM). An in vitro nitric oxide (NO) assay was performed to select compounds for in vivo anti-inflammatory activity evaluation. SF13 scavenged the NO level to a maximum of 85% at 50 µM concentration, followed by SF1 and SF2. Based on the NO scavenging assay results, in vivo anti-inflammatory studies were also performed and the results showed significant activity compared to the standard, acetylsalicylic acid (ASA).

A multi-disciplinary, multimodal approach for the management of vascular anomalies.

OBJECTIVE: Vascular anomalies are a diverse group of lesions, ranging from simple to complex, disfiguring anomalies. Our objective was to diagnose and provide comprehensive treatment to patients presenting with vascular anomalies, using a multi-disciplinary approach involving dermatologists, plastic surgeons, radiologists and pediatric surgeons. METHODS: Patients presenting with vascular anomalies to The Indus Hospital, Karachi, from January 2017 to March 2019 were enrolled, using a pre-defined questionnaire. Assessment, diagnostic work up, management and clinical and photographic follow up was maintained to monitor outcomes. RESULTS: One hundred eighty seven patients with a mean age of 4.6 years, (females 62%) were enrolled. Diagnoses included vascular tumors (n=89, 47.6%), lymphatic malformations (n=38, 20.3%), capillary malformations (n=19, 10%), venous malformations (n=16, 8.5%), arterio-venous malformations (n=14, 7.5%) and mixed anomalies (n=11, 5.9%). Treatment modalities, in isolation or combination, included oral propranolol, topical timolol, pulsed dye laser and intra-lesional sclerotherapy. Mean follow up was in 7.1 months, with 27 patients achieving treatment completion. 26 children were lost to follow-up. CONCLUSIONS: Vascular anomalies have mostly been managed successfully at VAC using single or multimodal treatment. Increasingly complex anomalies can be handled using a multi-disciplinary approach. Establishment of VAC has facilitated many patients who were earlier considered as diagnostic and therapeutic challenges.