Thrombotic thrombocytopenic purpura - analysis of clinical features, laboratory characteristics and therapeutic outcome of 24 patients treated at a tertiary care center in Saudi Arabia

Objective: Thrombotic thrombocytopenic purpura [TTP] is a life-threatening disease. The primary aim was overall response rate [ORR] assessment in the treated patients

Methods: This retrospective study included 24 patients treated during 2006-2015. TTP patients with microangiopathic hemolysis [MAHA] and thrombocytopenia were included. We analyzed clinical features, laboratory characteristics and treatment outcomes of 24 TTP patients treated at our tertiary care center [KFMC]

Results: Twenty-four TTP patients [18 females; 6 males] had a mean age of 33.5 +/- 13.9 years; 22[91%] had neurologic features, 7[29%] fever, 10[42%] renal impairment; 4[20.83%] cardiac manifestations; 22[91.7%] had triad with additional neurologic abnormalities; only 2[8.2%] had pentad of TTP. Majority [54.16%] had idiopathic TTP. All patients received therapeutic plasma exchange [TPE]; 23[95.8%] received adjunctive corticosteroids and 13[54.2%] received rituximab either due to refractoriness to TPE on tilde day7, or earlier. Twenty-one out of 24 [87.5%] achieved complete remission [CR] without any subsequent relapse. At 22 months [median, range 1-113], 20 patients [83.3%] are alive at the time of report. Three patients died during acute episode because of sever disease or delayed treatment and one died in CR

Conclusion: TPE, steroids and or rituximab was very effective in preventing high risk of mortality and achieving durable CR in 87.5% of patients. More awareness is needed for early diagnosis and early referral to centers with appropriate tertiary care facilities

Blurred vision as the sole initial presentation of disseminated lung malignancy

Symptomatic choroidal metastasis [CM] is a rare presenting feature of disseminated lung malignancy. Detection of the primary malignancy usually precedes ocular findings. We report a rare case of blurred vision secondary to bilateral CM as the sole initial manifestation of disseminated lung malignancy in a female patient. Pulmonary symptoms appeared 2 months later. She received radiotherapy and palliative chemotherapy and survived for 7 months after the initial presentation.

Distal penile fasciocutaneous flap for stricture disease of anterior urethra

To develop curative treatment for complex stricture disease involving anterior urethra. Twenty patients with a median age of 35 years with history of repeated optical internal urethrotomies >5 underwent surgery. Stricture site and length were evaluated by Retrograde and Antegrade urethrogram. Stricture calibration was done by retrograde ureteric catheter insertion of variable sizes. Onlay or tubular was flap applied over partial or blind stricture respectively ranging from 5cm to 15cm. The period of follow up was 24 months. Complications like patchy necrosis of penile skin [3] external meatal stenosis [2], Fistula [1] stricture at anastomotic site [1] and post void dribbling [5] were noted. The patient's quality of life improved following surgery. Distal penile fasciocutaneous flap is an effective tool for recurrent stricture disease involving anterior urethra

Blood and Body Fluid Exposure Related Knowledge, Attitude and Practices of Hospital Based Health Care Providers in United Arab Emirates

OBJECTIVES: Knowledge, attitudes, and practices of healthcare providers related to occupational exposure to bloodborne pathogens were assessed in a tertiary-care hospital in Middle East. METHODS: A cross-sectional study was undertaken using a self-administered questionnaire based on 3 paired (infectivity known vs. not known-suspected) case studies. Only 17 out of 230 respondents had an exposure in the 12 months prior to the survey and of these, only 2 had complied fully with the hospital's exposure reporting policy. RESULTS: In the paired case studies, the theoretical responses of participating health professionals showed a greater preference for initiating self-directed treatment with antivirals or immunisation rather than complying with the hospital protocol, when the patient was known to be infected. The differences in practice when exposed to a patient with suspected blood pathogens compared to patient known to be infected was statistically significant (p < 0.001) in all 3 paired cases. Failure to test an infected patient's blood meant that an adequate risk assessment and appropriate secondary prevention could not be performed, and reflected the unwillingness to report the occupational exposure. CONCLUSION: Therefore, the study demonstrated that healthcare providers opted to treat themselves when exposed to patient with infectious disease, rather than comply with the hospital reporting and assessment protocol.

Cold agglutinin disease in fibrolamellar hepatocellular carcinoma: a rare association with a rare cancer variant

Cold agglutinin disease [CAD] is a rare autoimmune hemolytic anemia. Although it can occur secondary to lymphoproliferative disorders and autoimmune or infectious diseases, CAD is rarely reported as secondary to solid tumors. We report a case of a woman aged 18 years diagnosed with a well-differentiated hepatocellular carcinoma of the fibrolamellar subtype, who was shown to have CAD also. Her general condition, including CAD, improved after targeted therapy with sorafenib for the hepatocellular carcinoma and only conservative measures for the CAD that consisted of avoidance of cold. In summary, although it is an extremely rare association and less common than lymphoproliferative disorders, CAD can be associated with solid tumors

Anesthetic management of a patient with hypertrophic obstructive cardiomyopathy undergoing modified radical mastectomy

Hypertrophic obstructive cardiomyopathy [HOCM] is a rare genetic disorder characterized by left ventricular outflow tract [LVOT] obstruction. Clinical presentation ranges from absence of symptoms to sudden death. Our 60 year old patient scheduled for left modified radical mastectomy had HOCM since seventeen years with severe LVOT obstruction and mitral regurgitation. An implantable cardioverter defibrillator [ICD] and permanent pacemaker [PPM] was inserted 15 months earlier for ventricular tachycardia. Anesthetic management of these patients presents considerable challenges and requires maintenance of desired hemodynamic parameters and management of specific complications. Factors like tachycardia, hypovolemia, vasodilation and increased cardiac contractility leads to exacerbation of the obstruction. In our patient there was the additional consideration of the ICD which required to be turned off during surgery with full provision for external defibrillation. We managed to successfully maintain the desired hemodynamics throughout the surgery and the patient was discharged home on the seventh postoperative day

Effect of macular photocoagulation on visual acuity of Omani patients with clinically significant macular edema

The aim of this study was to determine the effect of macular laser treatment on the visual acuity [VA] of Omani diabetic patients with clinically significant macular edema [CSME]. Visual outcome was also correlated with duration and control of diabetes and presence or absence of hypertension and hyperlipidemia. This is a retrospective noncomparative cohort study involving 101 eyes of 72 Omani diabetic patients. Change in VA was determined using Snellen's VA chart. The mean duration of follow-up was approximately 21 months [range, 16-24 months]. 29.7% of the patients maintained their vision, 35.6% showed improvement, whereas 34.7% showed a decrease in their vision. Positive visual outcome showed a statistically significant direct relationship with tight control of diabetes and absence of hypertension and an inverse relationship with the duration of diabetes. Presence of hyperlipedemia did not show a statistically significant relationship with positive visual outcome. However, it showed a trend to better visual outcome in the absence of hyperlipedemia. Peak incidence of macular edema was seen at the age of 52.3 years. Macular photocoagulation was found to be an effective method of treatment for CSME among Omani diabetic patients, which has resulted in a positive visual outcome in 65.3% of the patients [stable and improved vision]. Effective control of diabetes, duration of diabetes, and hypertension are the factors which influence the postlaser visual outcome

Spontaneous rupture of amyloid spleen

Primary systemic amyloidosis [AL] is characterized by the overproduction of immunoglobulin light chain proteins by a monoclonal, terminally differentiated B-lymphocyte or plasma cell clone. The free immunoglobulin light chains are deposited in an abnormal conformation as amyloid in a variety of organs in the body [1]. Splenic involvement is seen in 4 -13% of cases, however amyloidosis causing spontaneous rupture is very rare with only 31 cases reported so far. We report a case of 60-year old, suffering from Primary AL amyloidosis and multiple myeloma involving the liver, spleen and GI tract, who suddenly collapsed and developed rapid abdominal distension. CT scan suggested rupture of the spleen. She underwent emergency Splenectomy and had an uneventful recovery. It has been suggested that the deposition of amyloid in the basement membrane of the vascular endothelium leads to increased gap between junctions and leading to vascular fragility and propensity to rupture. The amyloid can also lead to defective interaction with platelets and predispose to spontaneous haemorrhage. Thus in a case of occult haemorrhage with Amyloidosis, diagnosis of rupture of spleen should be considered and CT scan should be performed if the patient is haemodynamically stable. Splenectomy is the ideal treatment for ruptured spleen

Immune factors in breast milk: a study and review

An established source of energy, vitamins, minerals, growth factors, enzymes and hormones, only recent key research has also documented the ability of mothers milk to reduce in infants the incidence of diarrhea, RTI, UTI, otitis media, neonatal septicemia, necrotizing enterocolitis, Crohn's disease, allergic disorders, SIDS, type I diabetes, and lymphoma. However, the apparently scanty data from Pakistan on relevant antimicrobial constituents has encouraged this evaluation of selected immune factors in the early milk of some Karachi lactating mothers. Milk specimens expressed in sterile containers from lactating women 36-48 hours after parturition were scrutinized for DLC, TIBC and iron, and its centrifuged supernatants for IgA, IgG, IgM, CRP, C3, C4, and Secretor status; blood was drawn for Hb% and ABO[H]/Rh grouping. A total of 25 colostrums, eleven of which were collected in January 2000 at Karachi's Imam General Hospital and 14 from voluntary lactating women 36-48 hours after parturition in the maternity ward of the city's Civil Hospital during Feb-Dec 2003, were processed at Dr. Essa's Lab and Diagnostic Centre, Karachi. All donors had uncomplicated pregnancies, and no signs of mastitis at the time of sampling. TIBC ranged from 283 to a high 795ug/dL in one mother with IDDM; CRP was mildly elevated [10-12 mg/L] in 22 subjects; IgG was not detectable in any specimen, but IgM was present [20-455 mg/dL], and sIgA, the major immunoglobulin in secretions, was significantly manifest [165-3090 mg/dL]. Both C3 [30-287 mg/dL] and C4 [7-54 mg/dL], key components of the complement cascade, were evident. The lowest sIgA amount [48 mg/dL] with only traces of IgM, C3 and C4 were noticed in our solitary blood group 'A2' subject incidentally found to be a non-Secretor. Selected antimicrobial factors in early breast milk expressed with informed consent from 25 Karachi lactating mothers were estimated. The ongoing study has hinted at individual variations from mother to mother in the blend of some of the pertinent constituents assessed, which encourages and necessitates additional sampling for confirmation

Infectious complications of invasive blood pressure monitoring in open heart surgery patients

To analyze the incidence of infectious complications of invasive blood pressure monitoring. Study was conducted at the Anaesthesia Department of Punjab Institute of Cardiology, Lahore. Design: Arterial catheter tip of 100 adult patient was sent for culture. Both right and left radial arteries were used for cannulation. Outcome Studied: Mean duration of arterial cannulation, any evidence of local or systemic infection, incidence of positive catheter tip culture and types of organism grown were noted. Positive arterial tip culture was seen in 31 patients. Staph epidermidis was the most common organism being seen in 17 patients. Mean duration of cannulation was 34.24 +/- 09.54 hours post operatively. None of these developed any local or systemic evidence of infection. Adherence to the aseptic technique during insertion and maintenance of arterial connulas is very important to prevent the infectious complications related to the procedure. Minimum handling of the whole monitoring system and limiting the duration of cannulation will certainly be helpful in this regard

Risk of developing right bundle branch block [R. B. B. B.] and complete heart block [C. H. B.] during pulmonary artery catheterization in open heart surgery patients

To analyze the incidence and associated haemodynamic disturbances of a new Right Bundle Branch Block [R.B.B.B] induced by Pulmonary Artery Catheter [P.A.C] with a particular emphasis in patients with preexisting Left Bundle Branch Block [L.B.B.B.] in a prospective manner. To establish whether prophylactic placement of a ventricular pacemaker in patients with pre existing L.B.B.B. during pulmonary artery cotheterization is necessary. The study was conducted at the department of Anaesthesiology and intensive care of the Aga Khan University Hospital, Karachi. Pulmonary artery catheterization was performed in 125 adult patients who underwent various open heart surgical procedures. Outcome studied: Incidence of new R.B.B.B induced by P.A.C. and its associated haemodynamic disturbances with a particular emphasis in patients with a pre existing L.B.B.B. was recorded. New R.B.B.B. occurred in 13 [10.4%] patients while none of the 1 1 patients with a preexisting R.B.B.B. developed C.H.B. during the procedure. Development of new block was not associated with any haemodynamic disturbance. Prophylactic temporary pacing is recommended in patients having a pre-existing R.B.B.B. before placement of P.A.C. in previous studies. However, our study does not show that previous R.B.B.B. is at risk of developing complete heart block [C.H.B.] during the procedure. In view of grave consequences of C.H.B. It is recommended that facilities of pacing should be on board in case it happens

Complications of pulmonary artery catheterization: a prospective study in open heart surgery patients

Pulmonary artery was catheterized in 125 adult patients undergoing various open-heart surgical procedures for perioperative haemodynamic monitoring. A standard approach to right internal jugular vein was used in all patients. Complications noted were related either to central venous access, pulmonary artery catheter [PAC] threading and during stay of catheter in the pulmonary artery. Post-operatively, X-ray chest was done in every patient and tip of the catheter was sent for culture once the PAC was removed. There was 8.8 percent incidence of inadvertent carotid artery puncture. Pneumothorax occurred in one patient. Arrhythmias occurred in 49.6 percent of the patients. New right bundle branch block occurred in 10.4 percent of the patients, while none of the 11 patients with a pre-existing left bundle branch block developed complete heart block during pulmonary artery catheterization. There was no incidence of pulmonary artery rupture associated with the use of PAC. There was a 9.6 percent incidence of PAC colonization, which was seen on culture of catheter tip. This investigation revealed a low incidence of morbidity associated with the use of PAC

Alport's syndrome, an undiagnosed entity with different modes of inheritance and review of literature

We report here three cases of presumed Alport's Syndrome from different families presenting with mild to moderate renal failure. Two of these were teenage girls, while the third was a teenage boy. We confirmed positive family history in all cases and the mode of inheritance seemed to be Autosomal dominant, X-linked and Autosomal recessive among the three families. Sensori-neural deafness and bilateral anterior lenticonus was found in all of them. Renal biopsy was done in two cases. Histopathological changes on light microscopy were suggestive of Alport's syndrome. Electrone microscopy could not be done due to lack of facilities. Family counseling had a little impact, as apparently health family members declined to undergo screening procedures due to apprehension of having the similar disease. Hemodialysis is well-tolerated and kidney transplantation prolong renal and patient's survival. This paper highlights the fact that the condition is rather under diagnosed in our population and needs to be considered where appropriate

Simultaneous bilateral percutaneous nephrolithotomy in Pakistan

Twenty three simultaneous bilateral nephrolithotomy cases performed in a remote area of the country are presented. The method was applied to the properly selected patients, considering the size, shape and position of the bilateral kidney stones. Minimum age of the patient was 4 years and maximum 70 years. In our opinion simultaneous bilateral percutaneous nephrolithotomy [SBPN] is the treatment of choice in properly selected cases with bilateral renal stones. It is even beneficial in cases who have dense and staghorn stones where Extracorporeal Shockwave Lithotripsy [ESWL] does not work. It is advantageous for the patient to be operated upon through SBPN as compared to consecutive percutaneous operations or open nephrolithotomy. This procedure is less time consuming for the urologist. Risk of operative and postoperative complications is negligible and hospital stay is short. The patient can resume his daily routine life at the earliest and it is cost effective procedure for him

Ectopic placement of central venous catheter, importance of x-ray chest

A case of malpositioning of central venous catheter which was judged to be correctly placed on clinical criteria is being presented. Abberant positioning was picked up on X-ray chest

Combined carotid endarterectomy and myocardial revascularization - anaesthetic management

Coronary and carotid artery disease frequently coexist. When a patient who requires myocardial revascularization is found to have significant carotid artery disease as well it raises the question that whether simultaneous or staged operations should be done. Anaesthetic management of a case of simultaneous myocardial revascularization and carotid endarterectomy is being presented

Endonephropexy

Targeted endonephropexy was performed in eight patients with renal ptosis at our department in Pakistan from Jan. 1993 till Mar. 1996 with satifactory results. Seven were male and one patient was female with the complication of pain or heaviness in the ipsilateral flank. Two patients complained of palpable mobile mass in the abdomen which disappeared on pressing. On ultrasonic examination kidney of the concerned side was found to be lower than the normal position which was confirmed by standing IVU examination. IVU examination also revealed tortuous ureter on the side of disease. One patient also had an accompanying stone. Operation consisted of puncture and dilatation of channel through lower calyx and advanced as is being mentioned in the text. Control IVU examination was performed after wound healing and was repeated after two months of operation. All the patients were symptom free after the operation. Standing x-ray films after the contrast material injection showed elevation of kidney with straight ureter. The idea of endonephropexy came from the observation that scar of nephrostomy channel is quite sufficient to hold kidney at its place. It was applied to fix the kidney at required level

Lipids in biliary lithogenesis

Serum and biliary lipoproteins, total cholesterol [Tc] and triglycerides [TG] were measured in patients with gallstones and in those without gallstones. Serum and biliary LDLc, TG and Tc were significantly higher [P < 0.001] in cases having gall stones than those without stones while HDLc were low [P < 0.001] in those with stones. No difference was found in very low density lipoproteins [VLDLc] in the two groups. Present data showed that there is a statistically significant correlation of serum and biliary lipoproteins specifically LDLc and HDLc [r = +.67 and r = +.56]. This report shows that serum HDLc [67.42%] and LDLc [70.28%] play a more critical role in comparison to total cholesterol [59.43%] and triglyceride [57.15%] levels in the formation of gallstone

Surgical removal of nasopharyngeal angiofibroma following super selective embolization [SSE]

Juvenile Nasopharyngeal Angiofibroma is a common clinical entity in Pakistan. Surgery is the treatment of choice in most centers. Radiotherapy is reserved for intracranial extensions only. Intraoperative blood loss associated with the surgical removal of these vascular tumours was brought down to 150-300 c.c. [Au: 200c.c.], follwing Superselective Embolization in this series of 25 cases. Blood transfusion was found to be unnecessary after the initial experience. No complication of embolization was noted in these patients. Two patients have returned with minor recurrence within a period of 24 months after initial surgery. This could be a disadvantage of S.S.E., as the tumour mass may have shrunken and yet a portion may have remained from resection to regrow at a later date