Biomedical subjects
Publications and source records attributed to S Budhiraja.
A six-year-old female patient presenting with a swelling in the infraumbilical part of the abdomen, bulging out on straining, was diagnosed to have pseudoexstrophy bladder. The urinary tract was normal. The patient had bifid clitoris. There was no other associated malformation. Surgical repair of abdominal wall defect was done successfully. A new classification of exstrophy variants is proposed.
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A simple technique of intraoperative decompression and colostomy in neonates with a massively distended abdomen is described. With this technique, the handling of distended bowel becomes easier and peritoneal contamination by meconeum is also prevented.
A retrospective study of 11 cases of choledochal cyst over a period of 10 years is presented. There were 8 females and 3 males in a ratio of 2.67:1. The classical triad of jaundice, mass and abdominal pain was seen in none of our patients. Ultrasonography was diagnostic in 9 out of 11 (80%) patients. Surgical procedures performed were, complete excision of the cyst with Roux en Y hepaticojejunostomy (nine cases), internal drainage of the cyst (one case) and excision of the extrahepatic cyst with Roux en Y hepaticojejunostomy in one case of Type IV choledochal cyst. One patient died in the immediate postoperative period. Others have remained well upto 10 years follow-up. We recommend total cyst excision with hepaticojejunostomy as the treatment of choice.
A 4 year old girl with progressive distension of abdomen and previous history of blunt abdominal trauma was found to have bile ascites due to left hepatic duct (LDH) injury. The management with a brief review of literature is discussed below.
A 10 year retrospective study of 45 cases of cystic lymphangioma (CL) in children is presented. There were 25 females and 20 males. Age ranged from 6 months to 8 years. Common sites were involved in 38 and rare sites in 7 patients. Rare sites were--gluteal region (1), pelvis (1), retroperitoneum (1), mesentery (2), inguinal region (1) and inguinoscrotal region (1). The clinical presentation included sudden increase in size (25), lump abdomen (3), gluteal abscess (1), abdominal distension (1) and inguinal swelling (2). Diagnosis was established preoperatively in 38 cases, and after surgery and histopathology in 7 cases. Near total or subtotal excision was carried out in all cases. Facial nerve palsy (1) and recurrence (2) were the complications of surgery. The study is presented to highlight the occurrence of the cystic lymphangioma at rare sites to avoid diagnostic errors and unnecessary mutilating surgery.
A 10-year-old male with multiple small swellings over the shaft of the penis for 2 years was found to have multiple neurilemmomas on histopathologic examination. The case is reported in view of the extreme rarity of the entity.
Caudal duplication is a rare entity due to incomplete separation of mono-ovular twins. It is usually associated with various congenital malformations involving mainly the genitourinary and gastrointestinal tracts. We report such a case operated upon successfully in which there was no other associated anomaly.
An unusual case of isolated levocardia, duodenal obstruction, and malrotation with volvulus is reported. Three cases of situs inversus abdominus with malrotation with volvulus have been reported to date. The current case is the fourth case and only the second survivor in the series. In cases of situs inversus abdominus, because of inversion of viscera, the volvulus may occur in an anticlockwise direction and, thus, should be derotated in a clockwise direction.
We have previously demonstrated that gene expression of the hepatocyte nuclear factor 3alpha (HNF-3alpha) transcription factor is activated during retinoic-acid-induced differentiation of F9 embryonal carcinoma cells (A. Jacob et al. (1994). Nucleic Acids Res. 22, 2126-2133). We have extended these studies and now show that HNF-3alpha mRNA is induced approximately 6 h after addition of retinoic acid to the cells, peaks at 1 day postdifferentiation, and then declines to undetectable levels. Furthermore, HNF-3alpha induction occurs in the absence of de novo protein synthesis, suggesting that it is a primary target for retinoic acid action. In order to corroborate this hypothesis, we have mapped the cis-acting HNF-3alpha promoter site that mediates the retinoic acid response. DNA sequence analysis indicates that the HNF-3alpha promoter contains an authentic retinoic acid response element (RARE) of the DR5 class. As expected, this element is able to confer retinoic acid responsiveness to a heterologous promoter. In addition, the HNF-3alpha-specific RARE is able to interact with various retinoic acid receptor heterodimers of the RAR/RXR type. Since HNF-3alpha is induced early during mammalian neurogenesis, our data shed new light on the connection between retinoic-acid-mediated HNF-3alpha activation and establishment of the neuronal phenotype.
INTRODUCTION: The role of hepatitis C virus (HCV) infection in fulminant hepatitis (FH) is poorly understood and the available data are conflicting. We have examined the aetiological role of HCV in 50 consecutive patients with sporadic FH by employing serology and reverse transcription-polymerase chain reaction (RT-PCR). MATERIALS AND METHODS: A total of 50 consecutive patients with sporadic FH were included. After an initial clinical and biochemical assessment, tests were performed for detection of HBsAg, IgM anti-HBc, IgM anti-HAV, IgM anti-HEV and anti-HCV. RT-PCR was carried out for detection of HCV RNA in sera of all the patients and in post mortem liver biopsy tissue of 20 subjects, using primers selected from the conserved 5' non-coding region of the HCV genome. RESULTS: Hepatitis E virus (HEV) was found to be the most common viral infection (21/50; 42%) followed by HBV (14/50; 28%), HCV (7/50; 14%) and HAV (2/50; 4%). No viral markers could be detected in nine patients (18%) and multiple infections were seen in seven (14%). Of the seven subjects who tested positive for HCV-related markers, two had both anti-HCV and HCV RNA, three had HCV RNA alone and the remaining two had anti-HCV alone. Interestingly, all the HCV-infected subjects were co-infected with other hepatotropic viruses and the most common co-infecting agent was found to be HBV (5/7). Liver tissue was available in 20 cases and HCV RNA was detected in three of them. All of these patients were also positive for the viral genome in their serum samples. Comparison of the biological attributes of HCV-positive and HCV-negative cases revealed that haemorrhagic symptomatology (haematemesis, melaena and purpurae) was significantly more common, prothrombin time more deranged and mortality was much higher in the former group. The overall mortality was 68% and the most common cause of death was cerebral oedema (70.6%). No significant correlation was observed between mortality and the duration of the icterus-encephalopathy interval. The study included a total of 21 pregnant females; HEV infection was found to be significantly greater in this group and was associated with a higher mortality rate. CONCLUSIONS: The results clearly suggest that HCV is not an important aetiological factor for FH in North India. However, it may act as a co-factor in the development of FH leading to a higher mortality. HEV appears to contribute substantially to the causation of sporadic FH in India and advanced stage pregnancy is a potential risk factor for HEV-induced FH and high rate of mortality. Our study also suggests that the length of the icterus-encephalopathy period may not have significant prognostic implications in Indian patients with FH.
A 2-year-old male with a perineal swelling, covered with normal skin without any rugosities, was diagnosed as having an accessory scrotum on histopathology. There was no other congenital anomaly. The case is reported due to its rarity and unique clinical picture.
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We have investigated the regulation of transcription factors HNF-3alpha and HNF-3beta during the retinoic acid-mediated differentiation of mouse P19 cells. Retinoic acid treatment converts P19 stem cells into neurons and astrocytes and we have clearly shown that gene expression of both HNF-3alpha and HNF-3beta is activated during this process. HNF-3alpha transcription was detected 2 h after addition of retinoic acid and took place in the absence of de novo protein synthesis. This suggests that HNF-3alpha is a primary target for retinoic acid action. HNF-3alpha induction displays a biphasic profile and HNF-3alpha mRNA reaches maximal levels at 2 and 6 days postdifferentiation. Additional experiments strongly suggest that the second peak is due to HNF-3alpha induction in postmitotic neurons. P19 stem cells, on the other hand, do not contain any detectable HNF-3alpha mRNA. According to our studies, the retinoic acid-mediated induction of HNF-3alpha occurs at the level of transcriptional initiation and is conferred by distal promoter sequences. In comparison to HNF-3alpha, HNF-3beta induction is a subsequent event and detectable levels of HNF-3beta mRNA materialize approximately 1 day after addition of retinoic acid to P19 stem cells. Time course studies firmly demonstrate that HNF-3beta mRNA peaks at about 2 days postdifferentiation and then declines to virtually unreadable levels. This temporal pattern is consistent with HNF-3beta being a secondary target for retinoic acid. In analogy to HNF-3alpha, HNF-3beta activation also takes place at the level of transcriptional initiation. Recent studies implicate HNF-3alpha and HNF-3beta in early mammalian neurogenesis. The detection of HNF-3alpha/beta activation during P19 cell differentiation provides us with a convenient cell culture system to elucidate the induction mechanism and the precise role of both transcriptional regulators in the formation of neuronal cells.
Accessory scrotum is the rarest anomaly of the scrotum; less than two dozen cases have been reported. A case of accessory scrotum with imperforate anus is reported.
We have examined the mechanism of regulation of rRNA synthesis in mouse F9 teratocarcinoma cells that were induced to differentiate by retinoic acid and dibutyryl cAMP. Ribosomal RNA (rRNA) synthesis was significantly reduced during differentiation of F9 cells into parietal endoderm cells. Nuclear run-on assay revealed that the rRNA gene transcription rates were reduced in differentiated cells, and this phenomenon could be mimicked by in vitro transcription assay using nuclear extracts prepared from F9 stem and F9 parietal endoderm cells. Analysis of the DNA-binding activities of two RNA polymerase I (pol I) transcription factors E1BF/Ku and UBF revealed decreased affinity for their cognate recognition sequences. Immunoblot analysis showed a marked reduction in the amounts of E1BF/Ku and UBF in the differentiated cells. Analysis of the steady-state RNA levels for the smaller subunit of E1BF/Ku and for UBF in differentiating F9 cells revealed decreased mRNA synthesis and increase in message level for the differentiation-specific marker laminin B1 with progression of the differentiated status of the cells. This study has demonstrated that differentiation of mouse F9 teratocarcinoma cells into parietal endoderm cells leads to diminished rRNA synthesis, which may be mediated by reduced DNA-binding activities and amounts of at least two pol I transcription factors.
This is a retrospective study of 27 cases of congenital short colon with anorectal malformation (pouch colon syndrome) treated during the last 5 years (from January 1991 to December 1995). The radiological feature of enormously dilated colonic pouch occupying more than half the width of abdomen was diagnostic in almost all cases. Excision of pouch and end enterostomy was associated with maximum survival (92.3%) in good risk patients. Proximal diverting enterostomy with decompression and irrigation of pouch is a preferable alternative to pouch colostomy in poor risk patients. Tapering coloplasty and colostomy should be reserved only for types I and II cases of pouch colon.
BACKGROUND: Viral hepatitis is a major public health problem in India. AIM: To investigate the association of various hepatitis viruses in patients with acute liver diseases in north India. METHODS: One hundred and thirteen patients with acute viral hepatitis (AVH; n = 70) or fulminant hepatic failure (FHF; N = 43) were evaluated for the presence of hepatitis A, B, C and E virus infection. Hepatitis C virus (HCV) testing was done using second-generation anti-HCV ELISA test and reverse transcriptase polymerase chain reaction (PCR) for the detection of HCV RNA in the serum of patients with non-A, non-B (NANB) hepatitis. Detection of IgM anti-HEV antibody was done in patients found negative for the above viruses (n = 53). RESULTS: Hepatitis A and B viruses accounted for 3.5% and 42.5% of the 113 cases, respectively. HCV infection accounted for 12% of the NANB cases with AVH and 15.5% with FHF. PCR was more useful than serological tests for the detection of HCV infection. HEV infection accounted for 49% of the NANB, non-C cases with AVH and 25% with FHF; pregnant women with HEV infection had a fulminant course. No etiological agent could be established in 28.3% of cases. CONCLUSION: HEV is the most important cause of NANB hepatitis; hepatitis B virus is still a major concern, while HCV is not an important cause of acute viral liver disease in India.