Zain Majid

Hepatitis E presenting with thrombocytopaenia.

We hereby present the case of a 25-year-old man who presented at the emergency department of Civil Hospital Karachi, Pakistan with signs and symptoms of acute viral hepatitis. Serology tests revealed that the patient was suffering from hepatitis E viral (HEV) infection. Concurrently, the patient was also found to have thrombocytopaenia (TCP). His TCP became better after the resolution of his jaundice, with the patient requiring a transfusion of one mega unit of platelets. After ruling out other common causes of TCP and after a thorough literature search, we concluded that an immune-mediated mechanism secondary to HEV infection might have been the cause behind his low platelet counts. Hence, we propose considering the possibility of HEV infection in patients presenting with acute liver failure and TCP, irrespective of age, gender, and geographical location of the patient.

Herpes Zoster-Induced Ogilvie’s Syndrome

Ogilvies syndrome due to herpes zoster infection is a rare manifestation of VZV reactivation. The onset of rash of herpes zoster and the symptoms of intestinal obstruction can occur at different time intervals posing a significant diagnostic challenge resulting in avoidable surgical interventions. Herein, we describe a case of 35-year-old male who presented with 6-day history of constipation and colicky abdominal pain along with an exquisitely tender and vesicular skin eruption involving the T8–T11 dermatome. Abdominal X-ray and ultrasound revealed generalized gaseous distention of the large intestine with air up to the rectum consistent with paralytic ileus. Colonoscopy did not show any obstructing lesion. A diagnosis of Ogilvies syndrome associated with herpes zoster was made. He was conservatively managed with nasogastric decompression, IV fluids, and acyclovir. The patient had an uneventful recovery and was later discharged.

Ascaris lumbricoides and its invasion of the accessory cystic duct: An unusual presentation

Around the world, Ascaris lumbricoides is the most common helminthic infection. We describe the case of a 25-year-old woman, known to have had Ascaris infestation, presenting with abdominal pain, constipation and jaundice together with fever and tachycardia. There was tenderness in the right hypochondrium and liver function tests confirmed cholestatic jaundice. An abdominal ultrasound showed multiple linear echogenic foci in the distal small intestine along with cholelithiasis and a thick-walled gall bladder with a single stone compressing the common bile duct (Mirizzi syndrome). The patient underwent exploratory laparotomy; more than 100 worms were found inside the small intestine and they were removed by enterotomy and manual decompression. No worm could be palpated within the common bile duct (CBD). Cholecystectomy was performed, during which an accessory cystic duct was noted opening into the common bile duct superiorly to the main cystic duct. A 10 cm live worm was found coming out of it and was removed via forceps. Later on an endoscopic retrograde cholangio-pancreatogram (ERCP) showed a widened ampulla, a mildly dilated common bile duct, but without any filling defects. The patient made an uneventful postoperative recovery being discharged on the ninth day. Worms in an accessory cystic duct have not been report in the medical literature so far.

Harlequin ichthyosis in an infant born to a father with eczema.

Harlequin ichthyosis is characterized by thickening of the layer of the skin which contains keratin. Eczema is a chronic relapsing skin disorder which is also associated with disrupted epidermal barrier. We report the case of a 6-hour-old male patient who was brought to the neonatal intensive care unit of our hospital with crusting skin lesions all over the body, presence of a severe ectropion and deranged electrolytes. A diagnosis of harlequin ichthyosis was made, and the neonate was managed accordingly. However, the infant eventually expired on the seventh day of life. The infants father was a patient of eczema with a chronic relapsing course and was on oral steroid therapy. As per our knowledge, this is the first reported case of an infant with harlequin ichthyosis born to a father suffering from eczema. The similarities in the pathogenesis of the two diseases and the genetic mutation of filaggrin might suggest an association between the two conditions. Harlequin ichthyosis can hence be looked out for in infants born of parents with eczema.

The usage of complementary and alternative medicine in gastrointestinal patients visiting the outpatients’ department of a large tertiary care centre-views from Pakistan

Introduction The use of complementary and alternative medicine (CAM) has increased over the last few years, and an emergent data suggests that some CAM modalities may be helpful in addressing gastrointestinal (GI) conditions. Our aim was to find out the prevalence of such practices for GI condition amongst patients visiting an OPD of a large tertiary care centre of Karachi, Pakistan. Methods Patients visiting outpatient department of Hepatogastroenterology department at SIUT, Pakistan from March 2014 to March 2015, were included in this cross sectional study. A pre designed questionnaire was used that included the demographic data, primary disease of the patient, CAM modality used, reason for the use of CAM therapy and reasons for stopping it. Frequencies of different variables were computed using SPSS version 18. Results 906 patients were interviewed, out of which 52% (471) were males. The mean age at presentation was 39.81±12.4 years. 234 (25.8%) of the participants used one of the CAM modalities; Herbal medicine being most common one, seen in 122 (52.13%) followed by spiritual 61 (26%), and homeopathy 33 (14%). The duration of therapy was limited to six months in 161(68%), whereas 7 patients (2.9%) had prolonged duration of use of more than five years. Reasons for using CAM therapy included advice by family and friends in 66 patients (28%), personal will in 42 (17.94%), no benefit from allopathic treatment in 34 (14.5%), while high cost was the reason of use in 3(5%) of the patients. The most common reason for discontinuation of CAM was no benefit, seen in 113 patients (48.30%), followed by physicians advice in 32 (17%) patients, and side effects in 19 (8%). On the other hand 44 patients (18.80%) reported benefit from the therapy while 14 (5.9%) were still continuing with CAM modality. Among the CAM users 140 (60.09%) were un-educated or had primary education while CAM nonusers had 328 (47%) were either uneducated or had primary education only correlation reveals P value 0.004. Conclusion Significant numbers of patients used CAM therapy. A lower level of education was associated with increased usage of CAM while cost had no major impact on its usage.

SCREENING AND VACCINATION AGAINST HEPATITIS A IN PEDIATRIC CHRONIC LIVER DISEASE PATIENTS

Background: Superadded HAV infection in patients with chronic liver disease (CLD) or cirrhosis may result in further deterioration of liver functions causing acute on chronic liver failure. The Center for disease control (CDC) strongly recommends to vaccinate all CLD patients against HAV. Methods: All pediatric patients with chronic liver disease for >6 months, after informed consent from their parents were enrolled. HAV immunity was defined as the qualitative presence of HAV IgG antibody (?1.00 reactive) in the serum blood test. All patients with negative HAV IgG were subjected to HAV vaccination and adverse events were also noted. Results: A total of 30 pediatric CLD patients with a mean age of 9.3 ± 3.9 years were enrolled. Majority of participants were males n=19 (63.3%). The overall prevalence of positive HAV serology (IgG) was 86.7 %. Three participants with negative HAV serology received a series of two vaccines, 6 months apart and HAV IgG titers were checked at 4 weeks after the first and second dose of vaccination. Despite two doses of vaccine satisfactory titer levels could not be achieved. Conclusion: This is the first study from Pakistan in which pediatric CLD patients were evaluated for HAV exposure. Early vaccination is advisable in such patients as advanced liver disease patients may not achieve adequate titers.

Stauffer syndrome; new trends

Many malignancies of the human body can cause cholestasis, which are mainly due to the hepatic metastasis, obstruction of the bile duct or could be due to a paraneoplastic phenomenon (1). Renal cell carcinoma is associated with a wide range of paraneoplastic syndromes (1). Such a paraneoplastic phenomenon causing deranged liver function tests in a patient diagnosed with renal cell carcinoma is called Stauffer syndrome (2), first described in 1961 by Stauffer himself (1). Stauffer syndrome is predominantly characterized by a raised erythrocyte sedimentation rate (ESR), elevated alkaline phosphatase (ALP) and gamma-glutamyl transferase (GGT), raised alpha 2 globulins, increased platelet count, prolong prothrombin time (PT) and by the presence of hepatosplenomegaly on examination without any evidence of hepatic metastasis or the presence of jaundice (2,3). To diagnose this syndrome, three of the previously mentioned abnormalities must be present in the patient (4). Stauffer syndrome has also been seen in various other malignancies like prostatic carcinoma, lymphoproliferative diseases and bronchogenic carcinoma (5). Intrahepatic cholestasis without jaundice is the main findings that are commonly seen in Stauffer syndrome (1). Treatment for this condition is nephrectomy that cures the symptoms in more than half of the cases (6). Authors’ contribution All authors wrote the letter equally.

Implementation of a triage system in Pakistan: the need of the hour

In today’s world, an effective emergency department (ED) cannot function properly without having a triage system. Though available in various forms, this essentially categorises patients according to the seriousness of their condition and the priority of their need. Pakistan is yet to introduce this system and where it exists, it is in its infancy. A disaster-prone country such as ours, which is frequently faced with both man-made and natural calamities, could benefit tremendously from its introduction. The activity of EDs would be streamlined and precious lives would be saved.

IgG4-related disease; a newly recognized entity with expanding spectrum

IgG4-related disease (IgG4RD) represents a newly recognized group of diseases affecting a wide spectrum of organs and systems of the human body. The disease has certain characteristic gross, morphologic, immunohistochemical and serological features. Its accurate diagnosis is important for optimal treatment and prognostication. There is a strong need for clinicopathological correlation to arrive at the correct diagnosis of this condition. Strict criteria must be used for identifying unusual cases of the disease in unusual locations. Key point IgG4-related disease (IgG4RD) represents a newly recognized group of diseases affecting a wide spectrum of organs and systems of the human body. Citation: Majid Z, Asif J, Khan S, Mubarak M, Masood I. IgG4-related disease; a newly recognized entity with expanding spectrum. Immunopathol Persa. 2017;3(2):e13.

Appendicitis with appendicular atresia: a rare presentation

Acute appendicitis is the most common acute surgical condition; making appendectomy the most commonly performed emergency surgical procedure in the world. Anomalies of the appendix are relatively uncommon. However, their presence may alter the course of pre-operative diagnosis and the surgical treatment provided, leading to medico-legal issues in certain cases as well. We hereby present the case of a 17 year-old female who had the suggestive signs, symptoms and investigations of appendicular lump. She was managed according to the Ochsner-Sherren regimen and then underwent interval open appendectomy 6 weeks later. During the procedure, the findings of a 5 cm long appendix were noted. The base of the appendix was attached to the caecum, however there was complete mucosal discontinuity between the base and the remaining portion of the appendix. A fibrous strand connected the two blind ending parts together. After thorough literature search, the authors concluded that this is only the fourth reported case of appendicular atresia ever to have been reported. Considering the rarity of this finding we feel this could be of valuable interest to surgeons and readers alike