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Objectives To study the incidence, clinical manifestations, and genetic spectrum of primary immunodefciency diseases (PID)/inborn errors of immunity (IEI) in a tertiary care hospital in Southern India. Methods A retrospective analysis of all patients with a clinical suspicion of PID/IEI seen at a tertiary care hospital was performed. All patients had at least one or more warning signs of PID. Serum immunoglobulin levels and other targeted investigations were performed as warranted by the clinical presentation. All families with suspected PID were counseled and ofered genetic testing. Results A total of 225 children were evaluated for PID during the study period of 6 y. Fifty-six of them did not meet the European Society of Immunodefciencies (ESID) criteria (working defnition of clinical diagnosis) and were excluded. An IEI was found in 30/49 (61.2%) patients. The most frequent reason for referral was recurrent/unusual or serious infections (28%), or cytopenia (16%). Group IV diseases of immune dysregulation was the most common category (19%), followed by group III predominant antibody defciencies in 23/163 (14%), as per the International Union of Immunological Societies (IUIS) classifcation. Conclusions This study highlights the heterogeneity of the present cohort, the underuse of genetic tests, and eforts to provide optimal care for children with possible IEI in this center.
RESUMO
Background:In the current era of minimally invasive interventions, the mainstay of treatment of renal stones larger than 2cm is percutaneous nephrolithotomy (PCNL). PCNL underwent various evolutionary changes minimizing morbidity to the patients. We prospectively compared the outcome of tubeless PCNL (without nephrostomy drainage tube) to reduce the pain and discomfort caused by tube with standard PCNL in the treatment of renal stones.Methods:In this randomized control trial (RCT), we divided patients satisfying the inclusion criteria of consenting for trial, single access puncture, less than 3 stones each less than 3 cm, operative duration of less than 2 hours into two groups, standard PCNL (group 1) and tubeless PCNL (group 2) with 25 patients each. Randomization and group assignment were done after complete clearance of renal stones.Results:Patient‟s age, gender, sides of stone and stonesize were comparable between two groups (standard versus tubeless PCNL). Postoperative hemoglobin drop, bleeding, pyrexia, urine leak, and blood transfusion requirement did not show a statistically significant difference between the two groups. Analgesic requirement (190mg versus 80 mg of tramadol), operative duration (49.80 min versus 38.60 min), postoperative pain score (6/10 versus 3.64/10-visual analog scale) and duration of hospital stay (68.48 hours versus 41.12 hours) showed statistically significant difference favoring tubeless PCNL.Conclusions: Tubeless PCNL may be a safe, acceptable and effective modality of treatment for renal calculi in carefully selected patients comparing standard PCNL resulting in less operative duration, lower postoperative pain, reduced analgesic requirementand shorter hospital stay
RESUMO
Background: Third stage of labour is still the “sword of Damocle’s” hanging above an obstetrician , inspite of today’s advanced technologies and personal care .The importance in the management of this deadly stage lies in the anticipation of complications and being quick enough to treat them timely. Hence in such scenarios, B-lynch suture is most popular method in treatment of uterine atony during caesarean section. The objective is to study and evaluate the cases in which the B-Lynch suture was used to treat the uterine atony during caesarean section.Methods: A prospective randomized study consisting of 50 women with high risk factors for atonic pph during caesarean section were included as study group patients were subjected to B-Lynch suture application when conventional drugs failed to control PPH. Blood loss was measured using a measuring jar. The fall in Hb% and the need for blood transfusion, and the need for hysterectomy as a last resort to save the life was evaluated.Results: The average blood loss was 1490 ml and the majority of patients had reduction in Hb% from 1.1-1.5 gm% (52%), 36% of the patients did not receive any blood transfusion after B-Lynch suture, and in 80% of cases hysterectomy was avoided.Conclusions: Our study shows cases treated with B-Lynch procedure showed that it is an effective method of containing pph. It has the advantage of being applied easily and safely. It should be attempted when conservative management fails and before any radical surgery is considered.