Primary splenic hodgkin's disease in a patient with chronic granulomatous disease, a case report

Here we report a 20-year-old male, a known case of chronic granulomatous disease [CGD], who presented with fever and splenomegaly. After splenectomy, primary splenic Hodgkin's disease was diagnosed. Immunohistochemistry confirmed the diagnosis [positive CD15 and CD30]. With chemotherapy, his fever was subsided and now after 6 months, he is doing well. Although primary immune deficiencies have been reported to show an increased tendency to develop malignancies, until now there has been no report of a patient with CGD and Hodgkin's disease

Peri-operative high-dose V post-operative low dose steroid therapy in the management of biliary atresia: a preliminary report

The use of high-dose steroid therapy peri portoenterostomy may have a positive impact on the frequency of cholangitis and survival rate. A prospective study was conducted on two groups of patients [less than three months of age] suffering from biliary atresia from 1999 to 2005. The patients in group I [G I] were managed peri-operatively by high-dose methylprednisolone while the other group [G II] received low dose methylprednisolone only post-operatively [2mg/k/day for 1 month]. Infants in GI [n = 30] received methylprednisolone for 3 successive days before operation [10-8-6mg/kg/day], and 10 mg/k at the day of operation respectively. Thereafter the dose was tapered in the next successive 6 days by 8, 6, 5, 4, 3, and 2 mg/kg/day and continued for one month. Seventy two infants with biliary atresia were operated [39 girls and 33 boys]. Twenty-six of the 30 patients [86%] in G I became jaundice-free within 90 days after portoenterostomy while only seven [15%] of the 42 patients in G II had normal bilirubin [P < 0.0001]. Episodes of postoperative cholangitis in G I were 20% [6 of 30], and 53% [24 of 42] in G II [P < 0.005]. The difference in 3-year survival rate between the two groups is also remarkable: Eighty seven percent [26 of 30] in GI versus 29% [13 of 45] in G II [P < 0.005]. Death related to biliary atresia occurred in 1 [3.3%] patient in GI compared with 12 [29%] patients in G II [p < 0.005]. These results provide strong evidence that peri-operative high dose steroid therapy is not only safe in this patients population, but because of its anti-inflammatory and cholerrhetic effects has a positive impact on preventing recurrent cholangitis, and ultimately survival

CIDP and achalasia: two manifestations of a disease or coincidental association

Chronic inflammatory demyelinating polyneuroradiculopathy [CIDP] is an immune mediated disorder characterized by progressive developing or relapsing symmetrical motor or sensory symptoms in more than one limb over a period of two months. Achalasia, as a primary esophageal motility disorder, is also characterized by increasing the tone of lower esophageal sphincter, absence or incomplete sphincter relaxation in response to swallowing, loss of esophageal peristalsis and rising intra-esophageal pressure. Herein, a case of CIDP, dysmotility- like symptoms, and achalasia is presented

Plasma concentrations of oral metronidazole in the early post-laparotomy periods

The bioavailability of metronidazole after oral ingestion, in ordinary conditions, is quite similar to intravenous administration of the drug. However, the degree of its absorption is not known in the early periods after laparotomy. To determine plasma levels of metronidazole in pediatric patients following elective abdominal surgery. The study group was comprised of 25 pediatric patients with mean age of 8.25 years. They took 10 mg/kg metronidazole [ingested or taken via nasogastric tube] a few hours after operation, followed by every eight hours for a total of three successive doses. We obtained blood samples 1-2 hours after each drug intake. The mean +/- SD of plasma drug concentrations after the first, second and third doses were 1.43 +/- 0.81, 6.26 +/- 3.86 and 10.21 +/- 4.28 micro g/ml, respectively, showing a significant rise after each dose [p<0.001]. The majority of patients [84%] obtained a level equal to, or above the minimal bactericidal concentration [MBC] after the third dose. Ninety-two and 96% of patients achieved the plasma minimal inhibitory concentration or higher following the second and third doses, respectively, as compared to 4% after the first dose [p<0.00001]. Absorption of oral metronidazole after elective laparotomy is disturbed only temporarily, as in the majority of patients the drug attains an acceptable level before the second post-operative day. Therefore, parenteral metronidazole therapy, if necessary, is recommended only during the first 24 hours, and it may be replaced by oral preparation afterward

Hypothalamo-pituitary response before and after surgical stress [splenectomy] in Thalassemic patients

Multiple endocrinopathy is a common manifestation in thalassemia. Although the response of the stress hormones to induced hypoglycemia has been studied in these patients, the impact of surgical stress is not yet determined. The hypothalamo-pituitary-adrenal axis of 27 thalassemic patients [TP], [4-15 years old [y.]] admitted for splenectomy, was evaluated before and after surgical stress during 1996-8. Blood samples for measurement of ACTH, cortisol, growth hormone [GH], thyroid stimulating hormone [TSH] and prolactin [PRL] were taken a day before and also approximately two hours after surgical insult. For comparison, 22 non-thalassemic patients [NTP] [3.5-14 y.] admitted for elective laparotomy, were selected as the control group. The cortisol response after surgical stress was significantly higher than baseline in both the TP [17.4 +/- 6.3 vs 30.81 +/- 11.49 micro g/dl; P<0.001] and the NTP [20.65 +/- 9.1 vs 36.87 +/- 11.08 micro g/dl; P<0.001]. NTP showed a significant elevation of ACTH upon surgical stress [P<0.001], while the difference between pre- and post-stress ACTH was not statistically significant in TP [P=0.123]. However, the ACTH concentration before operation in TP. was significantly higher than that of NTP [P<0.042] with no remarkable difference after surgical stress between the two groups [P=0.261]. GH increased significantly after operation in TP and NTP [P< 0.016 and <0.05, respectively]. A significant change in TSH [P< 0.03] and PRL [P< 0.004] was also observed in TP after operation. The hypothalamo-pituitary as well as the pituitary-adrenal axes are usually intact and responsive in TP. It is concluded that, the remarkable increase in ACTH concentration before operation may be due to a decreased adrenal reserve. Thus, the possibility of primary partial adrenal insufficiency, particularly under stress situations, should be considered in every thalassemic patient