Jejunal angiomatoses causing small bowel obstruction in a patient with Down syndrome: a case report

Gastrointestinal vascular anomalies are extremely uncommon. We describe a patient with Down syndrome who presented with acute abdominal pain due to a mixed capillary and venous vascular malformation involving the proximal jejunum.     

A giant vascular hamartoma of the breast in a child

Vascular tumors of the breast are uncommon. Perilobular hemangiomas, the commonest histologic subtype of breast hemangiomas, constitute majority of microscopic, vascular mammary tumors. Macroscopic hemangiomas and vascular hamartomas of the breast are distinctly rare. We describe a giant, vascular hamartoma of the right breast in a five year old girl and discuss pertinent literature.     

Anhidrotic ectodermal dysplasia presenting as nasal myiasis

An interesting case of anhidrotic Ectoderml Dysplasia (A.E.D.) which came with epistaxix, headache and maggots in nose is presented. Histopathology of forearm shin, scalp and Exercise test helped to confirm the diagnosis, AED is an X-linked recessive condition and has no cure but symptomatic treatment along with heat protection and protection of eyes especially in summer season has a role to play in the management of these cases.     

Bilateral enhancing thalamic lesions in a 10 year old boy: case report

A young boy presented with monoparesis of the left arm. MRI disclosed bilateral enhancing thalamic lesions. Biopsy results and subsequent clinical history were most compatible with postinfectious or acute disseminated encephalomyelitis. This represents one of the first cases of acute disseminated encephalomyelitis affecting the thalami, established by biopsy. This uncommon disease entity is reviewed and how it may affect the deep grey matter is described.     

Contribution of alcohol abuse to cerebellar volume deficits in men with schizophrenia

BACKGROUND: It is controversial whether cerebellar tissue volume deficits occur in schizophrenia and, if so, what regions and tissue types are affected. Complicating such investigations is the high incidence of alcoholism comorbidity in patients with schizophrenia that itself can contribute to cerebellar abnormalities. METHOD: We studied 61 healthy men (control subjects), 25 men with alcoholism, 27 men with schizophrenia, and 19 men comorbid for schizophrenia and alcoholism with the use of magnetic resonance imaging. Cerebellar structures were outlined manually, tissue classification was determined statistically, and regional volumes were corrected for normal variation in head size and age. RESULTS: Patients with schizophrenia alone had enlarged fourth ventricles (1.5 SD relative to controls) but showed no cerebellar tissue volume deficits. The alcoholic group had gray and white matter vermian deficits (-0.5 SD), most prominent in anterior superior lobules, and gray matter hemisphere deficits (-0.8 SD), but not fourth ventricle enlargement. The comorbid group had cerebellar hemisphere (-1.3 SD) and vermian gray matter volume deficits (-0.7 SD) and fourth ventricular enlargement (1.6 SD); these abnormalities were greater than in either single-diagnosis group, despite significantly lower levels of alcohol consumption compared with the alcoholic group. Gray matter volume in the anterior superior vermis correlated with lifetime alcohol consumption in the schizophrenic and comorbid groups when combined. CONCLUSIONS: Cerebellar tissue volume deficits were detected in schizophrenia only when accompanied by alcoholism. By contrast, fourth ventricular enlargement occurred in schizophrenia even without alcoholism, although it was exacerbated by alcoholism. These findings support a model of cerebellar supersensitivity to alcohol-related tissue volume deficits in schizophrenia.     

BDNF blocks caspase-3 activation in neonatal hypoxia-ischemia

Hypoxic-ischemic (H-I) injury to the brain in the perinatal period often leads to significant long-term neurological deficits. In a model of neonatal H-I injury in postnatal day 7 rats, our previous data have shown that cell death with features of apoptosis is prominent between 6 and 24 h after H-I and that neurotrophins, particularly BDNF, can markedly protect against tissue loss. During brain development, caspase-3 is required for normal levels of programmed cell death. Utilizing an antibody specific for the activated form of caspase-3, CM1, we now show that caspase-3 is specifically activated in neuronal cell bodies and their processes beginning at 6 h and peaking 24 h following unilateral carotid ligation and exposure to hypoxia in postnatal day 7 rats. Caspase-3 activation began to occur in cortex at 6 h and in striatum and hippocampus at 12-18 h. Caspase-3 activation was also observed in developing oligodendrocytes. Intracerebroventricular injection of BDNF prior to H-I injury almost completely abolished evidence of H-I-induced caspase-3 activation in vivo. Utilizing a specific molecular marker of an apoptotic pathway, these findings demonstrate that H-I injury to the developing brain is a strong apoptotic stimulus leading to caspase-3 activation, that BDNF can block this process in vivo, and that the ability of BDNF to inhibit caspase activation and subsequent apoptosis likely accounts in large part for its protection against neuronal injury in this model.     

Distal radioulnar joint incongruity after shortening of the ulna

Coronal sections of the distal radioulnar joint (DRUJ) were studied in 13 preserved cadaveric wrists specimens before and after 2 mm of shortening of the ulna. The DRUJs were subclassified on the basis of the DRUJ angle and depth of the sigmoid notch. The changes in extent and location of area of contact and radioulnar separation were noted. Although reduction in area of contact occurred in every specimen and DRUJ type, the maximum radioulnar separation and reduction in area of contact occurred in the DRUJ type IB and IIB. This could be a possible factor producing point loading and subsequent occurrence of DRUJ remodelling in the long-term after shortening of the ulna.     

Autopsy study of maternal deaths.

OBJECTIVES: To obtain an insight into the underlying disorder or pathologies in different organs or systems, and to attempt clinicopathologic correlation in maternal deaths. METHODS: This is a retrospective study of 95 maternal autopsies done from 1993 to 2000 in Sassoon General Hospital, Pune, India. External examination, in situ examination, gross and microscopic examination was done in each case. The cause of death was arrived at after reviewing clinical details, available investigations, morphological findings, and clinicopathologic correlation. RESULTS: Ninety-five (45.02%) out of 211 maternal deaths were autopsied. Out of 95, there were 47 (49.5%) direct obstetric deaths, and 33 (34.7%) indirect obstetric deaths. Fifteen (15.8%) deaths were unrelated to pregnancy, 14 of which were due to infections. CONCLUSIONS: Hypertensive disorders associated with pregnancy (24.2%) and anemia (14.7%) were most common. In the hypertensive group, important findings were disseminated intravascular coagulation, hemorrhages in different organs and thromboemboli. Two cases were HIV seropositive. The autopsy helped to elucidate factors contributing to death and pathology in different organ systems.