RESUMO
H syndrome is an autosomal recessive multisystemic disease with a very low prevalence rate, characterized by indurated cutaneous hyperpigmentation, hypertrichosis, and various systemic manifestations. The syndrome is caused by mutations in SLC29A3 gene on chromosome 10q23, encoding for human equilibrative transporter 3 (hENT3). So far, only 100-120 patients with H syndrome have been described in the literature, with predominance among Indian, North-American, and Arab ethnicities. This case report describes the first one of H-syndrome rarities in African ethnicity, a 30-year-old Sudanese male misdiagnosed with rheumatoid arthritis. The patient exhibited more than 90% of the clinical characteristics of H syndrome including obesity, short stature, characteristic hyperpigmented, sclerotic cutaneous plaques with induration and hypertrichosis, inflammatory arthropathy, hallux valgus, flexion deformity of toes, exophthalmos, cardiac anomaly, hypogonadism, and splenomegaly and characteristic histologic findings of dermal fibrosis, histiocytosis, lymphoid aggregation, and vascular proliferation. H syndrome is an extremely rare autoinflammatory condition that has a complex constellation of pleiotropic manifestations with multisystemic involvement. And while further identification and better pathophysiological understanding of H syndrome are needed, physicians worldwide should be vigilant about the overlapping features of H syndrome with many other rheumatological, cutaneous, and genetic diseases.
RESUMO
BACKGROUND: Caudal analgesia is the most commonly used technique providing intra- and postoperative analgesia for various pediatric infraumbilical surgical procedures but with the disadvantage of short duration of action after single injection. Caudal dexamethasone and magnesium could offer significant analgesic benefits. We compared the analgesic effects and side-effects of dexamethasone or magnesium added to caudal ropivacaine in pediatric patients undergoing inguinal hernia repair. MATERIALS AND METHODS: A total of 105 (1-6 years) were randomly assigned into three groups in a double-blinded manner. After a standardized sevoflurane in oxygen anesthesia, each patient received a single caudal dose of ropivacaine 0.15% 1.5 mL/kg combined with either magnesium 50 mg in normal saline 1 mL (group RM), dexamethasone 0.1 mg/kg in normal saline 1 mL (group RD), or corresponding volume of normal saline (group R) according to group assignment. Postoperative analgesia, use of analgesics, and side-effects were assessed during the first 24 h. RESULTS: Addition of magnesium or dexamethasone to caudal ropivacaine significantly prolonged analgesia duration 8 (5-11) h and 12 (8-16) h, respectively compared with 4 (3-5) h with the use of ropivacaine alone. The incidence of postoperative rescue analgesia was significantly higher in group R compared with groups RM and RD. The time to 1(st) analgesic dose was significantly longer in groups RM and RD (500 ± 190 and 730 ± 260 min) respectively compared with group R (260 ± 65 min). Group R patients achieved significantly higher Children's Hospital of Eastern Ontario Pain Scale and Faces Legs Activity Cry Consolability scores (4(th) hourly) compared with groups RM and RD patients (8(th) and 12(th) hourly, respectively). CONCLUSION: The addition of dexamethasone or magnesium to caudal ropivacaine significantly prolonged the duration of postoperative analgesia in children undergoing inguinal hernia repair. Also the time to 1(st) analgesic dose was longer and the need for rescue postoperative analgesic was reduced and without increase in incidence of side effects.
