Migration and maturation pattern of fetal enteric ganglia: a study of 16 cases.

AIMS: To study the migration and developmental pattern of ganglion cells in fetuses aged 9-21 weeks, and to document whether the migration was occurring circumferentially equally in the entire axis or if there were discrepancies in different portions at the same level. SETTINGS AND DESIGN: The hypothesis regarding the pathogenesis of Hirschsprung's disease mainly revolves around two schools. One is the single gradient migration of ganglia and the other is a dual gradient migration theory. Understanding the embryological development of enteric ganglia is necessary to study the pathogenesis of intestinal innervation disorders. MATERIALS AND METHODS: We studied the development of intestinal ganglia in fetuses aged 9-21 weeks. Serial longitudinal sections from the colon were studied, the first one including the squamo-columnar junction, for the presence and the nature of ganglion cells with Hematoxylin and Eosin, and neurone-specific enolase immunostaining. Transverse sections from proximal gut were studied in a similar fashion. Thus, we evaluated the migration pattern as well as the nature of ganglia in the fetuses. We also measured the length of distal aganglionic segment in these growing fetuses. RESULTS: We noted that ganglion cells appear first in the myenteric plexus followed by deep and superficial submucous plexus. We also found evidences in favor of dual migration theory, and the distal aganglionic segment varies around the circumference of the rectal wall. CONCLUSIONS: We got evidences in support of a dual migration pattern of intestinal ganglion cells. The level of distal aganglionic segments when measured from squamo-columnar junction varied with the age of gestation and the length was incongruous. The description of distal aganglionic segment may help surgeons while taking biopsies or during operative procedures.

Massive scapular metastasis as a presenting feature of carcinoma of the lip.

Carcinoma of the lip is a slow-growing locally-advanced disease with low metastatic potential. Distant skeletal metastasis is reported very rarely and the vertebral column is the most common site. A 58-year-old male smoker presented with pain and massive swelling of the left scapula for six months. He also noticed a slowly-growing painful ulcerated lesion on the outer aspect of the right lower lip for last two years. Biopsy from the lip ulcer, as well as cytologic smears from the scapular mass, revealed features of squamous cell carcinoma. Though no neck node was detected, patient was found to have extensive skeletal metastasis on whole body bone scan. It is rare as well as clinically appealing to have such disfiguring symptomatic massive scapular metastasis as the chief presenting feature in carcinoma of the lip.

Synchronous multicentric giant cell tumor.

Multicentric giant cell tumors represent less than 1% of all giant cell tumors of bones. We report a case of multicentric giant cell tumors around both the knee joints in a mentally and physically challenged adult male that resulted in rapidly progressive painful swelling, restricted mobility and, ultimately, fixed deformity. These tumors had typical radiological appearance and the diagnosis was confirmed on histopathology.

Micro-albuminuria: a predictor of 7-day mortality in acute myocardial infarction.

A study was undertaken amog 80 non-diabetic patients of acute myocardial infarction (AMI) admitted within 24 hours of the onset of pain, to investigate the prevalence and significance of microalbuminuria (MA) as a predictor of in-hospital mortality, and also to correlate it with other well-established prognostic markers. Spot urinary albumin-to-creatinine ratio (ACR) was measured in first morning sample on day 1 (D1), day 4 (D4) and/or day 7 (D7). Haemodynamic status was assessed clinically by Killip's class and the ejection fraction was measured by echocardiography on D1, D4, and/or D7. Total 7 days mortality was recorded. MA (>30 microg/mg) was found in 95% of patients on 1,. In the group with higher value of MA (>100 microg/mg) on D1, there was significantly more deaths (p <0.01). Also there was significantly more deaths with static or increasing MA value from D1 to D4/D7 (p <0.01). Increasing or static MA had a positive correlation with deteriorating Killip's class in non-survivors, and also there was a correlation between decrease in left ventricular ejection fraction from D1 to D4/D7 and an increasing trend in MA over that period. Thus, MA was found to be a reliable predictor of short-term in-hospital mortality in AMI.

Prognostic factors in haemotoxic viper bite: analysis of data from a referral hospital.

To identify the predictors of severe outcomes in patients with haemotoxic viper bite certain independent variables were recorded like age, sex, ethnicity, bite sites, duration of prehospital period, prehospital treatment, onset of systemic bleeding, local oedema, ecchymoses, blister, hematocrit, platelet count, whole blood clotting time, serum electrolytes, renal function and urinary examination findings in 62 hoptalised patients. Forward stepwise logistic regression was used for analysis of the independent factors predicting outcomes. Systemic bleeding was the commonest complication (45%). On multivariate analysis, alteration in coagulation parameters (like thrombocytopenia and prolonged whole blood clotting time) predisposed to systemic bleeding while local factors like bite site, faulty tourniquet application, and herbal treatment were responsible for local complications. Factors like massive envenomation and delayed hospitalisation significantly contributed to major organ damage. Predictors of death were anuria and systemic bleeding. It is concluded that several epidemiological, clinical, and laboratory features predict adverse outcomes in haemotoxic viper bite.

A study on endocrine dysfunction in adult males with liver cirrhosis.

Over a period of two years, 72 adult males with liver cirrhosis of different aetiologies were studied in terms of clinical and biochemical evidence of endocrine dysfunctions related to hypothalamic-pituitary-gonadal axis and the thyroid status, and compared with 40 age-matched control subjects. With more advanced disease, a progressive fall in testosterone, leutinising hormone and triiodothyronine and a rise in oestradiol was observed. Severity of the liver disease determined by Child-Turcotte-Pugh class, rather than aetiology (alcoholic or postviral), was the chief determinant of such dysfunctions. The involvement was both central and peripheral, with only peripheral defects at gonadal level in early state but dysfunctions at both the levels in late stage of cirrhosis.

Sickle cell hepatopathy.

Sickle cell hepatopathy is a well-documented entity that ranges from the self-limiting hepatic right upper quadrant syndrome to the potentially lethal intrahepatic cholestasis and acute hepatic sequestration syndromes. We describe a 26-year-male with homozygous sickle cell disease who had this unique hepatic presentation and was documented to have characteristic findings of cholestasis, portal inflammation and sinusoidal dilatation on histopathology.

A study on endocrine dysfunction in thalassaemia.

The aim was to study the prevalence and severity of hormonal imbalance affecting growth, gonadal and thyroid function in thalassaemic patients and to find out whether any correlation exists between the degree of tissue iron-overload and several patients characteristics like age, gender, foetal haemoglobin (HbF) level, type of thalassaemia (beta or E-beta), and the presence of specific endocrine abnormality. Sixty-eight consecutive non-chelated, transfusion-dependent patients of beta and E-beta-thalassaemia with significant tissue iron overload (serum ferritin more than 2000 microg/l) were included. Standing height was noted and clinical features of hypogonadism were recorded. Insulin tolerance test was done to assess growth hormone reserve. Serum oestradiol, T3,T4,TSH were measured in fasting clotted sample, while pooled sera (from 3 consevutive morning samples) was used for testosterone, FSH and LH. Hypogonadism was the commonest abnormality, both in males (52.28%) and females (35.89%) followed by growth retardation (20.58%) and reduced growth hormone reserve (7.35%). There was no significant difference in the prevalence of endocrine dysfunction with regard to patient's age, gender, type of thalassaemia (beta or E-beta) amd HbF level. Hypogonadic females had a significantly elevated mean serum ferritin level. Subclinical hypothyroidism was present in 23.52% of patients, related to the duration of disease. No association was found between pituitarty and thyroid dysfunction.

Primary amyloidosis presenting as intrahepatic cholestasis.

Hepatic involvement in primary amyloidosis is not rare but is often clinically silent. A mild elevation of the serum alkaline phosphatase level and hepatomegaly are the most common findings. We report a case of primary amyloidosis in an adult male presenting with intrahepatic cholestasis where viral, drug, alcohol, and autoimmune etiologies were all excluded and the definite diagnosis was established by liver biopsy with Congo red staining. Subsequently the patient was found to have monoclonal light chain disorder.

Retinal vasculitis--an initial presentation of systemic lupus erythematosus.

Retinal vasculitis is an intra-ocular inflammatory condition with diverse aetiology and a rare manifestation of systemic lupus erythematosus, often associated with antiphospholipid antibodies. A 13 years male presented with fever for 20 days associated with photosensitive skin rashes. Three years back he had suffered painless, progressive dimness of vision. Then a clinical diagnosis of retinal vascular occlusion was made. Examination revealed his visual acuity was reduced to finger counting at 3 metres distance and extensive retinal vasculitis in the right eye. His serum antinuclear factor was positive in high titre. A final diagnosis was made as systemic lupus erythematosus. He was treated with methylprednisolone. His fever subsided, skin rashes healed and vision did not deteriorate further.

Panniculitis-like T-cell lymphoma with fatal termination.

A 58-year-old male presented with three weeks history of multiple subcutaneous nodules and fever. Histopathological feature of the nodule was characteristic of panniculitis. Three months later, the patient presented with carvical lymphadenopathy and compressive thoracic myelopathy and a diagnosis of diffuse mixed- cell lymphoma was established. Immunohistochemical study of subcutaneous lesions confirmed their T-cell origin. Chemotherapy was started but patient succumbed to his disease.